Professional Documents
Culture Documents
MANAGEMENT
OF STRABISMUS
FIFTH EDITION
With 852 illustrations
With love and gratitude
to my wife
Barbara
Preface to the fifth edition
The 33 years since the publication of the Atlas of around the world. These patients' consultations were
Strabismus Surgery has seen a steady growth in the made possible by ORBIS Telemedicine, Cyber-Sight
number of ophthalmologists specializing in the diag- that is supported by the server at Flight Safety in New
nosis and treatment of strabismus. Membership in the York and provided through the generosity of Mr.
American Association for Pediatric Ophthalmology Albert Ueltschi. By viewing these images and read-
and Strabismus has grown to more than 500 mem- ing the discussion that accompanies each, it is my
bers and the International Strabismological hope that the reader will by critical analysis along
Association boasts 300 members in 25 countries. with comparison of the patients seen in his or her clin-
Several journals deal exclusively or largely with the ic hone skills for a more effective personal approach
subject of strabismus and several high quality texts to the whole patient leading to better outcome.
have been published dealing with strabismus diagno- This book represents the 'full circle' in that it is
sis and management. Moreover, an explosion in the based on experience during a professional lifetime in
field of information technology has brought people the care of patients with strabismus. It is my hope
together and facilitated the distribution of information that what I have learned and then shared here will be
in a way not dreamed of when the first edition of this of use to those who follow.
book was written. As with any effort like this there are many
This fifth edition is renamed Surgical deserving people to thank. First among these is Mrs.
Management of Strabismus reflecting the fact that the Lynda Smallwood who more than any person is
major emphasis has shifted from a description of sur- responsible for this book being completed. She not
gical procedures to a discussion of why and how only typed the manuscript, but also assembled and
these procedures are used to treat the patient with formatted the entire book making it ready for the
strabismus. There have been relatively few substan- printer by simply submitting it in electronic format
tive changes in surgical technique. It is how these ready for duplication. Truly a monumental task!
techniques are applied including the 'what' and 'when' Others who worked hard and effectively are Leslie
which make up the bulk of this book. Abrams, Michelle Harmon, Gwen Kopecky, Sharon
A new concept that has influenced surgical man- Teal who so ably carried on the art work begun by
agement during the last decade is that of the pulleys Craig Gosling, and Ann Hammer who read the first
associated with the horizontal rectus muscles. Study three chapters. I am grateful to the many Cyber-Sight
of these structures made possible in part by new partners who submitted hundreds of cases, and to my
imaging techniques, but more importantly by meticu- fellow Cyber-Sight strabismus mentors, especially
lous study of anatomic specimens, has helped make Dan Neely who faithfully and expertly responded
the management of 'A' and 'V' more logical and effec- when called on. I thank all of my teachers, especial-
tive. ly Gunter von Noorden my mentor beginning in 1966
Included in this book are 135 strabismus cases and my friend for life. Most of all, I thank my wife
managed by me either in the clinic or by means of Barbara for her unselfish and unfailing support, and
consultation carried out by me and other mentors on my two daughters both of whom had the good sense
patients sent by dozens of partners from countries to marry outstanding strabismologists.
Eugene M. Helveston
i
From the
There have been several excellent texts on stra- the vista of strabismus surgery.
bismus including strabismus surgery in the past few For these reasons it seems appropriate at this
years, but developments have moved rapidly. Recent time to compile an up-to-date atlas of strabismus sur-
advances in technique have greatly expanded the gery. This atlas employs schematic drawings
options available to the strabismus surgeon. More designed to illustrate at each step only that anatomy
accurate diagnostic tests leading to a better under- significant to the step shown for easier orientation of
standing of the pathophysiology of strabismus and the reader. Procedures that I have found useful have
amblyopia have convinced some surgeons of the need been given emphasis; those that are controversial or
for surgery in infants as young as 5 months of age. that I have not found to be particularly helpful have
Improved anesthesia and an increasing boldness on been omitted. Some "favorite technique" may be
the part of the strabismus surgeon have led to outpa- omitted simply because I prefer an alternative choice;
tient extraocular muscle surgery in some instances those that I think should be avoided will be clearly
without patch and without ointment or drops. The labeled so.
limbal and cul-de-sac (or fornix) extraocular muscle No attempt will be made to give a set of surgical
exposure techniques have largely superceded the recipes that will result in a predetermined amount of
transconjunctival incision in the interpalpebral space straightening. Instead, general concepts leading to a
among younger surgeons. The retinal surgeon has philosophy for strabismus surgery will be presented.
opened new dimensions in the degree to which sub- My intent is that this atlas will be of help to the prac-
Tenons space may be explored. ticing strabismus surgeon and the resident in ophthal-
There have been several excellent texts on stra- mology by bringing together in one volume many
bismus including strabismus surgery in the past few techniques from a variety of sources for quick and
years, but developments have moved rapidly. Recent easy reference.
advances in technique have greatly expanded the Several people who assisted significantly in their
options available to the strabismus surgeon. More own way to make this atlas possible deserve my sin-
accurate diagnostic tests leading to a better under- cere thanks. Dr. Gunter K. von Noorden, teacher,
standing of the pathophysiology of strabismus and critic, and friend, introduced me to strabismus and to
amblyopia have convinced some surgeons of the need the pursuit of academic ophthalmology. Craig
for surgery in infants as young as 5 months of age. Gosling worked with industry and imagination on the
Improved anesthesia and an increasing boldness on illustrations, the heart of any atlas. My residents and
the part of the strabismus surgeon have led to outpa- many of my colleagues, in particular Drs. Marshall
tient extraocular muscle surgery in some instances M. Parks and Phillip Knapp, provided both stimulus
without patch and without ointment or drops. and direction.
New sutures, adhesives, muscle sleeves, and
implantation materials have proved useful innova-
tions. Globe fixation sutures, conjunctival recession
and relaxation procedures, forced duction and active
forced generation tests, as well as topical anesthesia
for extraocular muscle surgery, have greatly enlarged Eugene M. Helveston
iii
Contents
Section 1
v
Contents
Section 3
vi
Contents
vii
Section 1
Strabismus, recognized from the earliest times In 1816 Dulspech, an orthopedic surgeon, performed
because the eyes are readily observable, has changed tenotomy of the Achilles tendon as treatment for club
little in form throughout the years. However, its caus- foot with a technique not too different from strabis-
es are now well understood, and its treatment under- mus surgery. Gibson experimented with extraocular
goes regular revision and refinement. The condition,
at first, was considered to be a visitation of an evil
spirit and incurable. The earliest physicians suggest-
ed treatment with the only available methods--
potions, purification, and diet. In the seventh centu-
ry, masks designed to redirect the visual axes were
described. Curiously, variations of this type of treat-
ment are still advocated by some today in the form of
sector occlusion, despite 13 centuries of failure!
The practice of strabismus surgery began inaus-
piciously in 1739 with the efforts of John Taylor
(Figure 1). He is reported to have had ...a consider-
able amount of sense..., according to Stewart Duke-
Elder, who said Taylor undoubtedly recognized that
strabismus was a muscle abnormality that could be
treated by dividing the extraocular muscles.*
However, Taylors surgery was not successful. On
the contrary, Taylor is said to have been a showman
who may have only snipped the conjunctiva, patched
one eye, and left town before the results could be
assessed. By patching the unoperated eye and having
the operated eye take up fixation, Taylors procedure
would have given the appearance of being successful
since the operated eye would appear to have been
straightened. John Taylors position in history
appears to be one of ridicule rather than honor.
After Taylor, several surgeons contributed to
the body of knowledge that led to successful, scien-
tifically founded strabismus surgery. In 1752, Figure 1
Eschenbach made the comment each oculist dreams Abundant showmanship and fast carriage rather than
from time to time that it may be possible to dissect surgical skills were outstanding attributes of the first
completely or partially the offending rectus muscle. strabismus surgeon John Taylor (1703-1772), an itinerant
healer who is depicted in this eighteenth century drawing.
*Duke-Elder S and Wybar K: System of ophthalmology, Vol 6, Ocular motility and strabismus, St Louis, 1973, The CV Mosby Co.
von Noorden GK (ed): History of Strabismology, Belgium 2002, J. P. Wayenborgh
1
Chapter 1
2
History of strabismus surgery
Figure 4
A wide variety of tendon lengthening procedures provided a safeguard against overcorrection and could also be carried out
without sutures, The simple Z tenotomy (Blaskovics, 1912) is still used today for weakening an already maximally recessed
muscle (Chapter 4).
A OConnor E Abadie
B Bishop-Harmon F Verhoeff
C Blaskovics G Terrien
D von Graefe
Some modern surgeons are reporting satisfactory clinical results from tenotomy which does not cut across all fibers, but
more or less nicks the muscle. Biglan reports success treating small vertical deviations by doing incomplete tenotomy of
the superior rectus.
3
Chapter 1
Figure 5
The use of sutures added to the complexity and safety, if not the effectiveness, of tenotomy. Again, the principle was that
a lengthened tendon-muscle would result in reduced muscle pull and therefore produce a change in alignment, shifting the
globe away from the weakened muscle.
4
History of strabismus surgery
A B
Figure 6
A Sutures were also used to reapproximate myotomized B Prince (1887) was credited with describing recession
muscles to avoid producing more than the intended with the tendon actually sutured to the sclera. This
result. The sutures were not placed in the sclera. They procedure was the forerunner of more accurate and
were placed only in the cut ends of the muscle and reproducible surgery.
tendon. The initial use of sutures apparently was for C Worth (1904) described resection using sutures. This
overcorrected and not primary cases. muscle shortening was accomplished with or without
recession of the antagonist muscle.
Late in the nineteenth century, measured reces- advances in anesthesia. A glass of wine and a good
sion with reattachment of the muscle to the sclera was lunch followed by a head lock and a quick surgeon
carried out. Tendon and muscle tucking or plication; were early anesthesia. This was replaced by ponto-
muscle advancement procedures; and, later, resection caine, cocaine, and ether for general anesthesia, and
and advancement procedures were also performed later, a wide variety of modern agents were used.
(Figure 6). Supporting modern anesthetic agents are the exten-
Surgery of the oblique muscles began when sive and detailed monitoring devices that provide
inferior oblique weakening was carried out to treat instantaneous, real-time recordings of temperature,
myopia and asthenopia, not surprisingly without suc- respiration, electrocardiographic data, and oxygen
cess. The superior oblique muscle was considered by saturation.
some noli me tangere, loosely translated today as off Highlights of the history of strabismus sur-
limits, in the early twentieth century. However, all gery from its earliest beginnings in 1739 to the pres-
types of oblique surgery had been described by the ent are depicted in the following drawings. They
middle part of the twentieth century. Fink, in 1951, describe a wide variety of manipulations that sur-
said up to a few years ago, all corrective surgical geons employed to straighten the eyes by altering the
measures for the obliques were not considered feasi- state of the extraocular muscle (Figures 7-24). The
ble because of anatomical difficulty.* statement has been made, Those who do not learn
Along with oblique muscle surgery and vertical from history are destined to repeat it. Thus, some
rectus muscle recession and resection, extraocular surgical procedures have a tendency to be rediscov-
muscle transfer procedures were used for strabismus ered every generation or so by surgeons who may
caused by muscle paralysis. Although, at first, the have overlooked an earlier description. Nonetheless,
improved alignment after eye muscle transfer proce- strabismus surgery has followed a steady progression,
dures was thought to be due to postoperative neuro- with improvement through innovations in the surgical
logic reorientation, it became apparent from elec- technique, combined with better instruments, more
tromyographic studies that the effect of an extraocu- reliable sutures, safer anesthesia, and a better appre-
lar muscle transfer procedure was mediated through ciation on the part of the surgeon of neural, sensory,
mechanical factors. and mechanical factors.
Technical advances in strabismus surgery have
occurred along with, and in some ways because of,
*Fink WH: Surgery of the oblique muscles of the eye, St Louis, 1951, The CV Mosby Co.
5
Chapter 1
Figure 7
Most early recession procedures involved sutures entering and exiting the conjunctiva for added security and to facilitate
removal of silk sutures. Tenons capsule and the muscle capsule were incorporated in recessions and resections. The
muscle is cut between bites of the preplaced suture to avoid loss of the disconnected proximal muscle end. Externally
placed buttons or bolsters added additional support to the recessed muscle, facilitated suture adjustment, and made suture
removal easier.
Figure 8
Jamesons technique for recession included several sutures anchoring the muscle to the sclera. The external sutures could
be removed and loosened or tightened postoperatively if a significant overcorrection or undercorrection was produced.
6
History of strabismus surgery
Figure 9
A Early needles were round; had eyes for threading the B Jameson admonished surgeons to keep the needle in
suture; and in most cases, had a wire diameter greater view while passing it through the sclera, to avoid entering
than the thickness of the sclera. These factors made the the globe and creating what he described as panoph-
ophthalmic needle a formidable weapon. thalmia.
Figure 10
Peters whip stitch for securing the cut end of the muscle during recession of a rectus muscle is a sound technique that is
still used today.
7
Chapter 1
A B
Figure 11
A A simple tuck for muscle-tendon shortening (strengthen- B When performed near the limbus, tucking tended to pro-
ing) avoided the need for placing a needle through the duce unsightly bulk that could be cosmetically disfiguring.
sclera and produced a shortened muscle without remov-
ing a piece.
Figure 12
Pragnen used gold buttons as bolsters to eliminate cheese wiring of the externalized suture when carrying out resection of
a rectus muscle. This technique allowed postoperative adjustment of the length of the muscle.
Figure 13
Peters modification of the Bishop tucking instrument was
one of several elegant instruments that were designed to
facilitate the tucking procedure. Tucking of the extraocular
muscles was an early favorite muscle shortening strength-
ening procedure.
8
History of strabismus surgery
Figure 14
An ingenious technique, the OConnor cinch, produced minimal shortening of
a rectus muscle. A large, dull needle was used to weave a multiple-strand
suture through slips of the tendon or muscle. The greater the number of
threads, producing a bulkier suture and a greater diameter of thread, the
more shortening of the muscle is produced.
B
A
Figure 15
A Peter suggested fracturing the trochlea and then shifting B The lateral rectus was shifted for treatment of congenital
and attaching the superior oblique muscle to a point near absence of the inferior rectus.
the medial rectus insertion. This procedure was com-
bined with lateral rectus tenotomy or recession for treat-
ment of third cranial nerve palsy.
Figure 16
A transcutaneous approach to the superior oblique tendon
afforded exposure for tenectomy or tucking. Skin incisions
were commonly used for exposure of both superior and infe-
rior oblique muscles in the early twentieth century.
9
Chapter 1
A1 A2
B C
D E
Figure 17
A1 Wheeler exposed the inferior oblique muscle nasal to E Wheeler strengthened the superior oblique muscle by
the inferior rectus through a skin incision. advancing the central portion of the tendon. He recog-
A2 The inferior oblique muscle was then engaged with a nized that this procedure produced decreased elevation
in adduction. In addition, he recognized that shifting the
muscle hook.
tendon anteriorly produced intorsion and that posterior
B From that exposure the muscle could be weakened by fibers influenced depression of the globe, a concept later
myotomy. credited to Harada and Ito (Jpn J Ophthalmol 8:88,
C The muscle also could be shortened by a tuck. 1964).
D A curious inferior oblique strengthening procedure per-
formed by Wheeler was disinsertion of the muscle fol-
lowed by reinsertion of the cut end into the inferior
orbital rim periosteum.
10
History of strabismus surgery
Figure 18
Hughes and Bogart exposed the trochlea subperiosteally, freed it, and pushed it backward several millimeters to weaken
the superior oblique. This formidable procedure was accomplished with a transcutaneous approach. No reports of series
of patients having this procedure performed on them were described. It is unlikely that his procedure was done widely; it
indicates the imagination and ingenuity of the early strabismus surgeon. This procedure, when performed inadvertently
after use of the Lynch incision for exposure of the ethmoid sinus, can produce superior oblique underaction, creating the
clinical picture of superior oblique palsy which, in turn, results in diplopia for the unlucky patient.
Figure 19
White plicated the superior oblique tendon medial to the superior rectus using a tucking instrument. He advised placing the
tuck nearer the superior rectus and away from the pulley of the trochlea to avoid having the superior oblique tendon become
hung up in the trochlea.
Figure 20
McLean engaged the superior oblique tendon temporally and brought the redundant loop of tendon temporally. This technique
for tucking the superior oblique tendon was said to reduce the incidence of postoperative limitation of elevation in adduction
by avoiding the trochlea and by reducing the likelihood of adherence of the redundant loop of tendon to the superior rectus.
However, any superior oblique tuck will cause a Brown postoperatively unless the tuck is done in a loose tendon and will result
postoperatively in an equal and normal superior oblique traction test (see chapter 9).
11
Chapter 1
Figure 21
Based on a procedure originally suggested by McGuire, Fink described resection of
the superior oblique tendon after detaching the superior rectus muscle. Removal
of the superior rectus provided improved exposure of the superior oblique tendon.
Figure 22
Fink recessed the inferior oblique muscle using a recession localizer that had a 7 cm handle with 6 mm arms projecting at
90 degrees from each other and a 1 mm tip with a concavity to hold gentian violet for marking. The purpose of the instru-
ment was to allow for an accurate 8.0 mm recession of the inferior oblique muscle. By estimation the recession could be
reduced to 6.0 mm or increased to 10.0 mm.
Figure 23
Following the lead of Duane, White and
Brown weakened the inferior oblique by
disinsertion without reattachment, consider-
ing the procedure safe and effective.
12
History of strabismus surgery
A B1 B2
C D E
F G H
I J K
Figure 24
Several techniques have been described for extraocular muscle transfer. The underlying principle for each procedure,
except superior oblique tendon transfer, is to shift the pull of antagonist muscles to a point on the globe coinciding with the
insertion of the rectus muscle lying between. The procedures shown have been described by the following:
A Hummelsheim F Schillinger
B1 OConnor G Beren-Girard
B2 Modified OConnor H Jensen
I Uribe
C Wiener
J Knapp
D Peter
K Helveston*
E Hildreth
*done in the case of an absent medial rectus
13
Chapter 1
A limited number of manipulations can be per- in early literature dealing with strabismus surgery.
formed on an extraocular muscle. The muscle can be Modern strabismus surgery differs significantly from
shortened and then made to stretch, at first under ten- early strabismus surgery in its attention to detail in
sion, to the original preoperative distance between dealing with the orbital fascia and fat. Recent
origin and insertion while presumably being more description of the anatomy of the trochlea, the inferi-
effective; the muscle can be retroplaced with the or oblique, and especially of the muscle pulleys has
same length of muscle going between two points provided new insight for strabismus surgeons.
closer together than the original origin and insertion; These tissues are thought by some surgeons to
or a longer muscle connecting the original origin be as important as the muscle itself. This book will
and insertion can be created with myotomy or tenoto- emphasize the importance of technique in the belief
my. The insertion can be shifted to a new place on that the surgeon who exercises impeccable care when
the globe, retaining the same innervation but having dealing with all tissues encountered in strabismus sur-
an altered mechanical effect. gery will attain better results.
Recent innovations in strabismus surgery Some operations that have been suggested by
include change in the arc of contact produced by the older surgeons or a few contemporary surgeons are
Faden operation (retroequatorial myopexy or posteri- not mentioned even as an option in either the histori-
or fixation suture); denervation of the inferior oblique cal or current sections. These omissions indicate a
muscle; detachment of the rectus muscles with spar- bias, which I hope will not result in overlooking use-
ing of the anterior ciliary vessels; and toxin injection ful procedures. Only procedures of sufficient histori-
at the motor end-plate to weaken a muscles function cal interest or contemporary procedures that have
by interference with nerve transmission at the some rational basis will be described. When appro-
myoneural junction. Thermal disruption of selected priate, alternative techniques will be described.
eye muscle fibers has been described but has not yet The use of adhesives and plastic sleeves and
been proven successful. sheets or caps mentioned in previous editions now are
Early strabismus surgeons had an accurate mentioned here only as a part of the history of stra-
grasp of muscle function but little appreciation for bismus surgery. Like many of the procedures shown
orbital fascial anatomy. Numerous references to the in this chapter, these techniques sounded good, were
encountering of fat during strabismus surgery suggest tried, but now are relegated mostly to memory.
that even the best surgeons found orbital fat on a rou- For a more complete review of the history of
tine basis. Except for referring to cutting across the strabismology, the reader is advised to consult The
conjunctiva, Tenons capsule, and the muscle capsule, History of Strabismology edited by Gunter von
scarcely a comment on these fascial tissues is found Noorden (J. P. Wayenborgh, Belgium, 2002).
14
2
Surgical anatomy
Overview
The successful strabismus surgeon has a clear extraocular muscle surgery. The orbital fat must be
understanding of the anatomy of the extraocular mus- recognized, respected, and left undisturbed.
cles which move the eyes, and also of the fascia, fat, Lockwood's ligament and associated lower lid retrac-
nerves, and the blood supply of the eye and orbit. tors providing support to structures that influence the
Although the extraocular muscles, especially that part lower lid position and to the inferior oblique and infe-
anterior to the equator, are the focus of strabismus rior rectus muscles must be dealt with properly to
surgery, structures such as the conjunctiva, anterior avoid lower lid ptosis. Whitnall's ligament associated
Tenon's capsule, posterior Tenon's capsule (intermus- with the levator palpebri, while not ordinarily
cular membrane and muscle sheaths), and the newly- encountered in the course of strabismus surgery, plays
described pulleys influence both movement and an important part in surgery of the upper lid. The vor-
alignment of the eyes. The sum of these structures, tex veins, although often observed in contrast to
including those observed during the course of surgery orbital fat which is not, should not be disturbed.
and others never seen, work in concert actively and Much of the new information about the anato-
passively to support, guide, restrict, or modify move- my and physiology of the structures of the orbit
ment of the eyes both physiologically in health and comes from laboratory studies using ultrasonography,
pathologically in the case of strabismus. computerized tomography (CT), magnetic resonance
The surgeon must be concerned with the imaging (MRI), and histochemical techniques. Use of
mechanics of access to the operative site, first some of these in clinical practice can also aid in stra-
between the lids and then through the conjunctiva and bismus diagnosis and in the design and execution of
Tenon's capsule. A proper start is required if the sur- successful extraocular muscle surgery.
geon expects a successful conclusion. The location, The following material describing surgical
as well as the blood supply, innervation, and action of anatomy and functional physiology is intended to
each of the extraocular muscles, must be known, provide practical information for the strabismus sur-
including the contribution of each muscle's intrinsic geon.
blood supply to the nutrition of the anterior segment.
Scleral thickness, which varies according to location, Palpebral fissure size
must be taken into account especially when placing a The dimensions of the palpebral opening
needle into the sclera. increase nearly 50% in width and 20% in height
The action of each extraocular muscle is influ- between infancy and adulthood. Configuration varies
enced by the location of its insertion on the globe and with a persons physical and racial characteristics
by the location of its pulley (or equivalent structure), (Figure 1). The size and shape of the palpebral open-
in particular, those pulleys associated with the medial ing should be considered at the outset of extraocular
and lateral rectus muscles. A thorough understanding muscle surgery. To start with, a lid speculum appro-
of these anatomic relationships forms the basis for a priate to the size of the palpebral opening should be
logical mechanical approach to surgery on the used (Figure 2). In addition, the surgeon should
extraocular muscles, including recession, resection, expect to encounter more difficulty with exposure
transposition, transection, plication, union, and and suture placement in medial rectus recession and
chemodenervation. In addition, orbital structures sur- also in patients with a small palpebral fissure or
rounding the globe and extraocular muscles pro- deeply-set eyes. However, measured recession can be
foundly affect the design, execution, and results of
15
Chapter 2
16
Surgical anatomy
taking place primarily in the posterior part, the rela- undercorrection of congenital esotropia occurred in
tive position of the medial rectus insertion will move nearly 50% of cases when the maximum for medial
anteriorly, and therefore safely, with maturity. rectus recession was on the order of 5.5 mm from the
The point of measuring from the limbus is that insertion as was the rule in the 1960's. This under-
this provides a safe technique for performing a larger correction rate reduced immediately to approximate-
recession of the medial rectus, especially in those ly 10% in a series when medial rectus recession was
cases with medial rectus insertions closer to the lim- measured from the limbus. This technique of reces-
bus (Figure 6). Measurement from the limbus begs sion measured from the limbus, allowing larger but
the question, "Is the significant factor in correction of safe recessions, is now joined by larger recession
esotropia the size of medial rectus recession or the measured from the insertion. These larger recessions,
new position of the muscle on the globe?" The some 7 mm or more, have been implicated in a high-
answer may be that both play a role. We do know that er overcorrection rate.
A B
Figure 5
A The medial rectus of a 4-month-old measures B The muscle has been recessed 9.5 mm from the limbus
approximately 10 mm at the insertion. using a limbal incision.
Figure 6
In a 4-month-old with an axial length of 19.5 mm, a recession 9.5 mm from the limbus places the new insertion of the
medial rectus approximately at the equator.
17
Chapter 2
Pulleys
Around 1990, Joseph Demer and associates
began study of the anatomy and actions of the A
extraocular muscles using high resolution magnetic
resonance imaging on clinical patients and normal
subjects aided in some cases by the use of paramag-
netic MRI contrast agents (Figure 7, 8). This work,
along with detailed histologic and histochemical
study of human and monkey orbital tissue in the lab-
oratory, led to the following summation by the C
authors in 2002. "The resulting reexamination of
EOM (extraocular muscle) anatomy and physiology
has been so revealing as to suggest a fundamental
paradigm shift having broad basic and clinical impli-
cations."
Demer and associates conclusions are summa-
rized as follows: B
1. Orbital structures called pulleys are associ-
ated with each of the rectus muscles and the SAGGITAL
inferior oblique.
2. The pulleys receive the contractile force of Figure 7
The pulley zone is roughly at the junction of the middle
the extraocular muscles and inflect the paths and posterior third of the globe, similar to Listings plane
of the muscles in a "qualitatively similar (see page 32).
manner to the inflection of the superior A Trochlea of the superior oblique - inflection of the
oblique (SO) tendon path by the trochlea" superior oblique.
B Lockwood ligament - the pulley of inferior rectus -- the
(Figure 9). functional origin of the inferior oblique (the functional
3. The paths of the extraocular muscles posteri- insertion of the inferior oblique after distal myectomy).
or to the pulleys (between the pulleys and the C The pulley of the horizontal recti.
annulus of Zinn) remain constant regardless
of the position of the globe. There is no side
slip of the rectus muscles, (except in the case
of an abnormality of the pulley).
4 . The functional origin of the extraocular mus-
cles is at their pulleys (Figure 10).
5. The orbital one-half of the extraocular mus-
cle fibers insert into the pulley and the bulbar
one-half of extraocular muscle fibers pass
forward to attach to the globe at the muscles
insertion (Figure 11).
6. Only that portion of the extraocular muscle
anterior to the pulleys moves in the direction
of the globes movement (Figure 12).
7. Upward displacement of the lateral rectus
pulleys and downward displacement of the
medial rectus pulleys are associated with A
pattern. (Figure 13) Downward displacement CORONAL
of the lateral rectus pulleys and upward dis-
placement of the medial rectus pulleys are Figure 8
associated with V pattern (Figure 14). A Trochlea
8. Pulleys made up of collagen, elastin, and B Confluence of superior oblique tendon and superior
rectus sheath
richly innervated smooth muscle are situated C Lockwoods ligament
in the orbit in the area previously called D Pulley of the horizontal recti
check ligaments. They are not readily distin- E Whitnalls ligament
guishable clinically and require special tech- F Levator palpebri
niques to be seen in the laboratory.
9. Several other pulley abnormalities could be
associated with strabismus entities including:
18
Surgical anatomy
Figure 9
The pulleys which inflect the paths of the muscle.
A Medial rectus pulley
B Lateral rectus pulley
B
D
Figure 10
The functional origin of the rectus muscles is at the pulleys.
Figure 12
The muscle - tendon anterior to the pulley
A Passes straight in primary position
B Courses upward in upgaze
C Courses downward in downgaze
D The direction of the muscle posterior to the pulley does
not change during up and downgaze
Global
Orbital
Figure 11
The orbital fibers insert into the pulleys of the horizontal
recti and the global fibers insert into sclera.
19
Chapter 2
20
Surgical anatomy
Figure 16
V esotropia in a patient with antimongoloid palpebral
fissures.
21
Chapter 2
Figure 17
A patient with myelomeningocele and a straight lower lid
margin simulating a mongoloid slant. This is a common
but unexplained finding in such patients.
Epicanthal folds
Epicanthal folds are present to some degree in
most infants and children during the first few years of
life (Figure 18 A, B). These skin folds can create an
illusion of esotropia. Parents think one eye turns in
B
because no white can be seen medially, especially in
the adducted eye in lateral versions. Two techniques
can be used to relieve parental concern regarding
pseudoesotropia from epicanthus. First, the examin-
er demonstrates the centered pupillary reflexes with a
muscle light. Second, the examiner carefully pulls the
skin forward over the bridge of the nose to demon-
strate the straightening effect of exposing the medi-
al conjunctiva or white of the eye (Figure 19). It is Figure 18
still a good rule for the ophthalmologist presented A Epicanthal folds obscure the nasal conjunctiva in both
patients, giving the appearance of esotropia. However,
with an obvious case of pseudostrabismus to carry out
the light reflex is centered in the pupil in each case.
a complete eye examination, including cycloplegic This reflex indicates the presence of parallel pupillary
refraction and retinal examination. A medial skin fold axes and, therefore, straight eyes or absence of
sweeping upward from below is called epicanthus manifest strabismus. Cover testing must be performed
eventually to confirm the presence of parallel visual axes
inversus (Figure 20).
because a large angle kappa* could hide a small
Telecanthus, which is an increased interorbital manifest esodeviation.
distance, may be confused with epicanthus (Figure B Epicanthal folds are present, but the displaced pupillary
21). Normally, the intercanthal distance is about one- reflex in the right eye confirms the presence of a right
esotropia.
half the pupillary distance. Intercanthal separation in
excess of this suggests true telecanthus, but this diag- *Angle kappa is the angle formed by the pupillary axis and the visual
nosis must be confirmed by radiologic evaluation axis. A positive angle kappa is present when the visual axis is nasal
to the pupillary axis. This simulates exotropia and is common. A neg-
demonstrating a bony abnormality. Other midline ative angle kappa is present when the visual axis is temporal to the
facial abnormalities, especially clefting of a facial pupillary axis. This simulates esotropia and is much less common
than positive angle kappa.
structure in the presence of telecanthus, should raise
the suspicion of defects at the base of the skull includ-
ing encephalocele. These patients also may have optic
nerve anomalies ranging from hypoplasia to morning
glory disk or even may be missing a medial rectus
muscle.
22
Surgical anatomy
A A
B B
Figure 19 Figure 21
A Centered pupillary light reflex A This patient demonstrates telecanthus with an
B The straightening effect of exposing more white nasal- interorbital dimension clearly more than one-half the
ly. (This is shown in an older patient because it is diffi- interpupillary distance and also an exotropia.
cult to photograph the younger child where the test is B This patient with telecanthus also has prominent
more effective.) epicanthal folds.
Conjunctiva
The bulbar conjunctiva, fused to the underlying
anterior Tenon's capsule, loosely covers the anterior
part of the globe from the fornices above and below
and from the canthi medially and laterally. The bul-
bar conjunctiva and anterior Tenon's capsule have
multiple, fine imbedded arterioles and veins. These
are branches of the anterior ciliary circulation and of
the marginal arcades of the vessels of the lids. Their
Figure 20 number and configuration vary from person to per-
A skin fold originating below and sweeping upward is called son. This circulation furnishes a small but probably
epicanthus inversus. This deformity is frequently significant blood supply to the anterior segment. The
associated with blepharophimosis and ptosis. These three
deformities, which may be combined with telecanthus, fused conjunctiva and anterior Tenon's capsule attach
cause significant disfigurement and present a formidable firmly to the sclera at the limbus (Figure 22). The
therapeutic challenge. combined conjuctiva and underlying anterior Tenon's
capsule is thick and has substance in infancy and
childhood but becomes much thinner and more fri-
able in adulthood and old age.
The plica semilunaris is a fold in the conjunctiva
located far medially in the palpebral fissure and is
mostly below the midline. The caruncle, located just
medial to the plica, is about 3 mm in diameter, cov-
ered with squamous epithelium, and often contains
small hairs (Figure 23). The relationship of the plica
and caruncle to each other and to the palpebral fissure
is an important cosmetic factor in strabismus surgery.
When repairing the conjunctiva, care should be taken
not to alter the position of these structures. It is par-
23
Chapter 2
24
Surgical anatomy
A B
C D
Fat Pad
Figure 24
A The shallow lower fornix with a visible fat pad beneath D Saggital section of the orbit shows the relationships of the
conjunctiva vertical rectus muscles, fat compartments, oblique
B The deep upper fornix with no visible fat under con- muscles, orbital septa, and lids. It should be noted that on
junctiva the superior aspect of the globe, the extraconal fat lies
C The inferior fat pad shown frontally above the levator palpebrae and behind the orbital
septum. Surgery on the superior oblique muscle is not
associated with extraconal fat because of the barrier of the
levator palpebrae muscle. Pulling the lower lid down
allows the inferior fat pad to prolapse.
anterior Tenon's capsule, exposing extraconal fat, disruption of the pulleys of the medial and lateral rec-
there will be no or reduced pulley effect on the eye tus muscles.
muscle. This will result in up and down slip of the While extraocular muscle surgery is performed
muscle relative to the globe. It is not practical or even beneath anterior Tenon's capsule, it is done within the
logical in the usual strabismus surgery to free pulleys plane of posterior Tenon's capsule. The intermuscu-
outside anterior Tenon's capsule, but this could be lar membrane part of posterior Tenon's capsule must
done for special need. Eye muscle surgery is routine- be fenestrated in order to place a muscle hook behind
ly performed entirely inside anterior Tenon's capsule the insertion of a rectus muscle (Figure 26 A-D).
with no fat exposure (Figure 25 A-C). How much more dissection is done in the intermus-
Posterior Tenon's capsule, composed of the cular membrane beyond the minimum required to
muscle's capsule and the intermuscular membrane, gain access to the muscle is the decision of the sur-
unites the rectus muscles in a ring around the globe. geon. It is probably wise to do as little cutting of pos-
The extent to which the intermuscular membrane is terior Tenon's capsule as is compatible with the con-
cut during surgery influences how far the rectus mus- duct of the surgical procedure intended. Retinal
cles, particularly the medial and to some extent the detachment surgery, in contrast to extraocular muscle
lateral, will retract during surgery. Dissection of pos- surgery, is carried out beneath posterior Tenon's cap-
terior Tenon's capsule far posteriorly leads to expo- sule. This enables a view of the scleral surface far
sure of intraconal fat, so called because it resides posteriorly to a point near the posterior ciliary vessels
inside the muscle cone. Excessive dissection of ante- and the optic nerve.
rior Tenon's capsule exposes extraconal fat and risks
25
Chapter 2
10
A
6, 4
26
Surgical anatomy
B C
Figure 26
A When the layer of fused conjunctiva-anterior Tenon's B Posterior Tenons capsule attaches to sclera at the
capsule is retracted, the muscle insertion in its sheath is muscles insertion and in the intermuscular space
exposed. Fibrous attachments are seen between the forming the spiral of Tillaux.
undersurface of anterior Tenons capsule and the outer C The muscle hook is placed in a hole created in
surface of the muscle. The fusion of the intermuscular intermuscular membrane adjacent to the muscle
membrane (posterior Tenon's capsule), as well as of the insertion and glides along bare sclera behind the rectus
muscle to the sclera, is apparent. This fusion of the muscle insertion and is exposed at the opposite muscle
intermuscular membrane to the sclera must be incised border with a snip incision.
before the bare sclera and subposterior Tenon's capsule continued.
space can be encountered. Only after entering
subposterior Tenon's capsule space can the insertion of
the rectus muscle be engaged cleanly on a muscle hook.
This is the free space used by the retina surgeon. The
tip of the scissors in the photo points to this free space.
27
Chapter 2
28
Surgical anatomy
* Although the lateral rectus insertion site is variable, it is not common to measure from the limbus for recession of this muscle.
29
Chapter 2
Head Head
A B
Head Head
C D
Head
Figure 29
A The superior rectus muscle seen through the intact D The insertion of the medial rectus muscle seen through
conjunctiva and anterior Tenons capsule. the intact conjunctiva.
B The insertion of the inferior rectus muscle seen through E The insertion of the lateral and inferior rectus muscles
the intact conjunctiva. Note fat pad. seen through the intact conjunctiva with the inferior
C The insertion of the lateral rectus muscle seen through temporal orbital fat pad seen just inside the lower lid
the intact conjunctiva. margin. The site of the incision for inferior oblique
exposure is shown. This view is shown from above.
30
Surgical anatomy
31
Chapter 2
description of the pulleys provides a useful concept globe, the insertion. The pulling effect on the globe
for appreciating Listings plane which appears to comes from the location of the pulley regardless of
coincide with the functional insertion of the rectus where the pull is initiated before it reaches the pulley.
muscles at the pulleys (Figure 31). This new post pulley concept replaces the earlier
held notion that the functional insertion of the
extraocular muscle was at the point of tangency with
the globe, which is anterior to the equator medially
A B and just behind the equator laterally. The new pulley
concept seems to be more compatible with the results
of strabismus management particularly dealing with
A and V patterns in comparison with those previ-
ously held (Figure 32).
Muscle forces
The extraocular muscles have a resting tension of
12 to 15 g. This tension increases to 40 to 50 g in the
agonist during a saccade. Tension in the antagonist
C
also increases somewhat during a saccade because of
the length tension effect and despite decreased inner-
vation. Maximum isometric contraction of an
extraocular muscle is approximately 125 g. The ten-
sion of the extraocular muscles when the eyes are
Figure 31 shifted away from the primary position and then sta-
A Saggital representation demonstrates how Listings bilized is greater than the tension in the primary posi-
plane coincides logically with the pulleys as the tion. When the eye is stabilized in any position; that
functional origin of the rectus muscles. is, not moving, tension is equal in opposing muscles.
B Frontal view of Listings plane
C Looking up to the left, the eyes move in Listings plane.
When the eyes are in a position away from the pri-
mary, static tension must be increased compared to
the primary or neutral position. This is to overcome
the passive forces of the orbital fascia which must be
Pulleys deflected to maintain position of the eye away from
The extraocular muscles initiate ocular move- the primary position. When the eye muscles are in
ment and then sustain a new position of the globe the resting state under general anesthesia, the eyes are
through a complex transfer of energy. The insertion more or less centered in the palpebral fissure or they
of each extraocular muscle on the globe acts on what may be slightly exotropic. If the eyes are passively
we tend to think of as a point-to-point basis. The moved from this position, they will spring back. This
actual muscle-globe relationship, however, is mediat- action is the basis for the spring back balance test of
ed through a complex arrangement of fascial attach- Jampolsky. This is a technique for evaluating the bal-
ments, including anterior and posterior Tenon's cap- ance of purely passive forces in ocular alignment and,
sule, conjunctiva, and numerous attachments between therefore, a guide to surgery in cases with mechanical
these structures. Initiation of the globe's movement restrictions.
can only be carried out by the action of the muscles. During pursuit movements, both eyes move at
This movement can be slowed or stopped by the pas- the same, usually moderate, speed, and in the same
sive fascial structures, including fat that surround the direction: right, left, up, down. While carrying out
globe and extraocular muscles. Movement of the vergence movements, eye movement is slower and in
globe stopped by passive fascial structures is useful opposite directions. During convergence (e.g. the
physiologically, or harmful in pathologic states. right eye moves to the left and the left eye to the
Normal ocular movements are stopped by mechanical right), both eyes are looking toward the nose, so to
factors in the extremes of abduction, adduction, ele- speak. The opposite takes place during divergence,
vation, and depression. These mechanical factors are although pure divergence amplitude is limited and
the check ligaments associated with the pulleys or occurs in the normal only when stimulated. When the
pulley-like structures that are formed by fascial con- eyes suddenly change fixation from one object to
densations between anterior Tenon's capsule and the another, a rapid movement or saccade takes place.
periorbita. In simple terms, the muscles act like a The two eyes normally move at the same speed and in
rope passing through a pulley on their way to attach- the same direction at speeds up to 250 to 400
ing to the globe. The pull of the muscle on the globe degrees/sec. This saccadic speed is reduced in a
occurs at the point where the muscle attaches to the paretic muscle more or less proportional to the degree
32
Surgical anatomy
Figure 32
A The medial rectus inserts between 3 and 6 mm from the C In the pathologic state, restriction in any of the inner
limbus. fascial structures around the globe may limit passive
B Pulley tissues also act as check ligaments at extremes ductions. In the presence of a normal agonist, these
of gaze. abnormalities usually result in increased intraocular
pressure and changes in the palpebral fissure.
33
Chapter 2
Figure 33
The configuration of structures passing through the optic foramen, superior and inferior orbital fissure, and annular ligament
of Zinn of the left eye.
34
Surgical anatomy
Overaction of an extraocular muscle is more The inferior oblique muscle was subjected to sur-
accurately described in most cases not as over exu- gery as early as 1841, but for myopia! By 1885, infe-
berance of the muscle but as underaction of the pas- rior oblique weakening for treatment of superior
sive checking tissue. Observation of clinical strabis- oblique palsy was done. The muscle was exposed
mus suggests that only cases of excess innervation through a skin incision and the muscle was cut medi-
such as occurs in the yoke muscle of a paretic muscle al to Lockwood's ligament. This technique persisted
can legitimately be called overaction. In spite of the into the middle of the twentieth century. After that,
obvious misnomer, the term overaction is firmly more attention was directed to weakening the muscle
implanted in the literature and the language of the distal to Lockwood's ligament nearer the insertion.
strabismologist. Currently the most effective techniques for inferior
oblique weakening include myotomy, myectomy, and
Surgical anatomy of the recession, placing the new insertion at various posi-
inferior oblique tions in the inferior temporal quadrant according to
The inferior oblique muscle is 36 mm long. It the surgeon's preference. Surgical techniques for
originates a few millimeters behind the medial end of inferior oblique weakening also vary in the extent to
the inferior orbital rim just lateral to the lacrimal which the inferior oblique is freed from its union with
fossa and proceeds posteriorly and temporally at an Lockwood's ligament and in the management of the
angle of 51 degrees with the frontal plane passing neuro-vascular bundle.
beneath the inferior rectus (between the inferior rec- In order to avoid surgery on the inferior oblique
tus and the floor of the orbit) (Figure 34). It inserts in some cases of overaction, Bielschowsky was said
beneath the inferior border of the lateral rectus mus- to have lowered the medial rectus. This portended
cle, approximately 12 mm from the insertion of the treatment of A and V pattern with vertical shift of
lateral rectus. The posterior extent of the inferior the rectus muscles and possibly the description of the
oblique insertion overlies a point 2 mm below and 2 pulleys of the rectus muscles whose anomalous loca-
mm lateral to the macula. The middle of the distal tion leads to vertical incomitance.
half of the muscle covers the inferior temporal vortex Recently, inferior oblique anterior transposition
vein. The blood vessels in the inferior oblique do not has been used for the treatment of inferior oblique
contribute to the blood supply of the anterior segment overaction especially when it is associated with dis-
of the globe. This muscle receives its innervation on sociated vertical deviation (DVD). The mechanism
its upper surface at the point where it passes beneath of action for this treatment of DVD has been
the lateral border of the inferior rectus, approximate- explained by Stager who demonstrated that the robust
ly 12 mm posterior to the lateral corner of the inser- neurovascular bundle of the inferior oblique is effec-
tion of the inferior rectus. The inferior oblique mus- tive in anchoring the transposed inferior oblique
cle is unique in its anatomic relationships. This mus- insertion. This is the same neurovascular bundle that
cle behaves as though it has two potential insertions was cut by Parks during the denervation and extirpa-
and two potential points of origin. Because the infe- tion procedure for maximum weakening of the inferi-
rior oblique is innervated near its middle, it may be or oblique.
weakened either proximal or distal to its point of Myectomy or large recession of the inferior
innervation. oblique distal to the muscles attachment at
A B
Figure 34
The inferior oblique (A) from in front and (B) from behind.
35
Chapter 2
Lockwood's ligament makes this attachment equiva- oblique. The descriptive term elevation in adduc-
lent to the new functional insertion (Figure 35). tion describes a condition where the inferior oblique
Although not done now, earlier procedures for weak- is responsible for elevation, not necessarily from its
ening the inferior oblique, which were carried out overacting but rather from the lack of checking from
nasal to the ligament of Lockwood, meant that the a weak (or absent) superior oblique. In defense of the
inferior oblique union with Lockwoods ligament term overaction of the inferior oblique, this term
became the functional origin. A procedure described also describes the extorsion and abduction caused by
by Stager and Weakley transected the inferior rectus the inferior oblique in cases of anomalous orbital
on both sides of Lockwood's ligament relying on a anatomy and/or upshift of the medial rectus pulleys
small segment of the middle of the muscle stabilized and in cases of deficient adduction.
by Lockwood's and the neurovascular bundle. In
cases of extirpation and denervation of the inferior Lockwoods ligament
oblique, a large myectomy of the distal inferior Lockwoods ligament may be compared to a
oblique is combined with transection of the neurovas- hammock supporting the globe (Figure 36). It forms
cular bundle. a dense condensation of tissue that engulfs the inferi-
The inferior oblique is unique among the or rectus and inferior oblique muscles beneath the
extraocular muscles in that, in many cases, weaken- globe. The attachment of Lockwoods ligament to the
ing of this muscle, even by extensive surgery, seems inferior oblique affects globe movement from the
to have relatively little effect on movement of the inferior oblique muscle when it contracts, even when
globe or alignment of the eyes. Even after large the inferior oblique is transected on both sides of
recession or myectomy, apparent overaction of the Lockwoods!
inferior oblique can persist. This is probably due to Attachments between Lockwoods ligament
horizontal rectus action from upward pulley displace- and neighboring muscle and fascial structures are
ment of the medial rectus. Also, in the relatively connected to the lower lid. This makes lower lid pto-
uncommon inferior oblique paresis, strabismus is sis a potential complication of inferior rectus reces-
much less than would occur after paresis of any of the sion (Figure 37). To avoid this, the inferior rectus
other muscles. Effective weakening of this muscle should be freed extensively during surgery. Guyton,
could be made more difficult because of the unique et. al., have recommended that Lockwoods ligament
anatomy. Likewise neurologically, the muscles be advanced when recession of the inferior rectus
innervation by the inferior branch of cranial nerve III muscle is carried out. When resection of the inferior
makes isolated paralysis rare. In contrast, the inferi- rectus is performed, persistent attachment of this
or oblique seems to overact commonly. But is muscle to Lockwoods ligament can cause just the
overaction the right term? Some think it is not, sug- opposite, a bothersome and cosmetically unaccept-
gesting that the preferred term would simply describe able elevation of the lower lid resulting in narrowing
appearance not etiology. The term "elevation in of the palpebral fissure. Freeing the inferior rectus
adduction," which replaces the Latin "strabismus sur- muscle from Lockwoods ligament also helps avoid
soadductorius," seems to be a valid description of this complication.
what has been called overaction of the inferior
Figure 35
The inferior oblique behaves as if it had two potential origins and two potential insertions because of its union with
Lockwood's ligament as it passes beneath the inferior rectus. In addition, at the mid-section of the inferior oblique is a stout
neurovascular bundle, described in detail by Stager and associates, which acts both as a restraining anchor and a source
of innervation.
36
Surgical anatomy
A B
Figure 36
A The ligament of Lockwood could be compared to a C The inferior fat pad is prominent and should not be
hammock supporting the globe. disturbed during surgery of the inferior rectus.
B The inferior oblique passes beneath the inferior rectus,
through Lockwoods ligament and orbital fat
approximately 12 - 14 mm from the limbus.
Figure 37
A saggital section of the complex anatomy of the orbit shows the intimate relationship of the inferior rectus, inferior oblique,
and Lockwoods ligament. This complex, in turn, is connected to the lower lid tarsus and inferior orbital septum. The
inferior extraconal fat protrudes farther forward compared to the extraconal fat of the superior globe. Recession of the
inferior rectus causes recession of the lower lid and widening of the fissure. Advancement or resection of the inferior rectus
causes narrowing of the palpebral fissure. Placement of the conjunctival incision too far from the limbus inferiorly can result
in disturbance of the extraconal fat compartment.
37
Chapter 2
Superior oblique
The superior oblique muscle has a muscular por-
tion and a tendinous portion, both of which are
approximately 30 mm long. The muscle portion orig-
inates superiorly and nasal to the ligament of Zinn at
the apex of the orbit and becomes tendinous 10 mm
before reaching the trochlea. The trochlea, a carti-
laginous saddle-shaped structure, is located at the
junction of the medial and superior orbital rim just
posterior to the orbital rim. The trochlea acts as a pul-
ley redirecting the course of the superior oblique ten-
don. approximately 54 degrees from the frontal plane.
The tendon passes beneath the superior rectus, insert-
ing under the lateral border of the superior rectus usu-
ally 5-7 mm posterior to the temporal superior rectus
insertion or approximately 13 mm from the limbus
(Figure 38). That portion of the superior oblique ten-
don passing beneath the superior rectus muscle is
attached to the undersurface of this muscle through
the common sheath of the superior rectus muscle. Figure 38
The superior oblique tendon is redirected to 54 from the
Therefore, to obtain an effective large recession of the frontal plane and passes posteriorly and temporally beneath
superior rectus muscle, it is logical to free it from the the superior rectus.
superior oblique tendon. A hang loose superior rectus
recession which is not secured at the intended rein-
sertion site may not accomplish the intended retro-
placement of the superior rectus muscle unless the
superior oblique - superior rectus attachment is freed.
The diameter of the superior oblique tendon
between the trochlea and the medial border of the
superior rectus is about 3 mm. The tendon is white,
surrounded by dense fascia, and lacking a discreet
tendon sheath. Because of this fascia, the superior
oblique tendon nasal to the superior rectus can be
somewhat difficult to identify when approached out-
side of anterior Tenons capsule. However, when
approached from the undersurface of anterior Tenons
capsule, the superior oblique tendon is an easily dis-
tinguishable structure (Figure 39). The nerve to the
superior oblique enters the muscular portion 26 mm Figure 39
From Parks MM, Helveston EM. Direct visualization of the
posterior to the trochlea. Blood vessels in the superi-
superior oblique tendon. Archives of Ophthalmology, 1970,
or oblique do not contribute to the blood supply of the 84:491-494. Used with permission.
anterior segment of the globe .
The insertion of the superior oblique is broad,
measuring on average 10.7 mm. The fibers at this
point are very thin and fuse with sclera in a manner
that makes the superior oblique insertion difficult to
distinguish from sclera. Only after carefully looking
in the area where the superior oblique should insert
can these fibers be seen coursing temporally and
slightly posterior. At times the surgeon must employ
a fine hook to carefully tease the insertion into view.
The insertion of the superior oblique has been shown
to be the most variable of any of the extraocular mus-
cles. While the tendon usually inserts at the lateral
border of the superior rectus muscle about 5 to 7 mm Figure 40
behind the superior rectus insertion, the superior The 'normal insertion of the superior oblique varies. The
insertion displaced medial to the superior rectus results in
oblique tendon can be found more anterior. In other
underaction.
38
Surgical anatomy
cases, the superior oblique can be found inserting at the sclera, as in hang-loose recession, an unpre-
the medial border of the superior rectus. This results dictable result may occur simply on the basis of the
in superior oblique underaction with excyclotropia anatomy. The union of the superior oblique tendon
because this nasal displacement reduces the torsional and superior rectus could pull the superior rectus for-
effect of the superior oblique. A wide range of other ward toward the limbus as the eye rotates from
anomalies is seen with the superior oblique tendon depression to the primary position (Figure 42).
from laxity causing congenital superior oblique Therefore, the hang-loose recession of the superior
palsy to absence of the reflected tendon. This spec- rectus muscle used in cases of dissociated vertical
trum of anomalies suggests a new way of classifying deviation lacks a sound anatomic basis.
congenital superior oblique palsy (see page 157). In order to recess the superior rectus more than 5
The superior oblique tendon joins with the or 6 mm with a hang-loose, the superior rectus
undersurface of the superior rectus muscle by a com- should be freed from the superior oblique. For a
mon muscle-tendon capsular attachment. This attach- recession as great as 10 mm as some claim with the
ment can be seen clearly when the superior rectus hang-loose, the superior rectus insertion must be
muscle has been detached from the globe and lifted behind the path of the superior oblique. Prieto Diaz
upward (Figure 41). The firmness of this attachment demonstrated with x-ray a 15 mm recession of the
varies, but these two structures do not appear to be superior rectus from the limbus in down gaze after
entirely free of any connection to each other in the hang-loose of the superior rectus. Would this reces-
normal state. As stated above, if recession of the sion be as large with the eye in primary position?
superior rectus is attempted without securing the
superior rectus at the intended site of reattachment to
Figure 41
A The superior oblique passes beneath the superior rectus B The superior oblique remains attached to the superior
rectus when the rectus is detached and pulled up.
39
Chapter 2
Figure 42
A When the eye is rotated downward, the superior rectus B When the eye returns to the primary position, the
is the intended distance in a very large hang loose superior rectus could be pulled forward, reducing the
recession even if the superior oblique tendon - superior amount of recession.
rectus union is intact.
40
Surgical anatomy
Whitnalls ligament
Whitnalls (superior transverse) ligament and the usually results. Therefore, it is safer to hook the supe-
superior oblique tendon in the trochlea have common rior oblique tendon under direct vision. This can be
fascial attachments at the orbital rim (Figure 43). If done between the nasal border of the superior rectus
the superior transverse ligament is weakened inadver- and the trochlea or an even safer place is at the inser-
tently while hooking the superior oblique tendon, tion. Whitnalls ligament acts as a clothesline, sus-
thereby weakening the medial horn of the levator pending the levator aponeurosis and the medial por-
muscle, ptosis of the nasal portion of the upper lid tion of the superior oblique tendon.
A B
Figure 43
A The relationship of Whitnalls ligament and the superior C Nasal ptosis right eye from disruption of Whitnalls
oblique tendon. Blind hooking the superior oblique ligament after hooking of the superior oblique tendon in a
tendon can damage Whitnalls, producing ptosis. blind sweep nasal to the superior rectus.
B Whitnalls ligament acts like a clothesline with orbital
structures suspended.
41
Chapter 2
Trochlea
The trochlea remained the largest undescribed the trochlea is the functional origin of the superior
portion of human anatomy until 1982. I began the oblique muscle (actually the superior oblique ten-
definitive study of the human trochlea with the origi- don).
nal exenteration specimen shown below that con- The tendon of the superior oblique can tele-
tained the superior oblique tendon, trochlea, and the scope inward toward the apex of the orbit approxi-
distal superior oblique muscle, all in their physiolog- mately 16 mm during maximum downgaze in adduc-
ic relationships* (Figure 44). The intact nature of the tion and telescope 16 mm outward in maximum
specimen allowed separation of the structures com- upgaze in adduction. Tendon movement cannot
prising the superior oblique complex as shown. This exceed these limits because the peripheral superior
specimen was carefully dissected and extensively oblique tendon fibers are attached to the trochlea.
recorded including videotaped images showing the The multiple fiber layers making up the superior
way in which the superior oblique tendon passed oblique tendon slide with a cumulative effect with
through or more accurately slid in a telescoping man- only the central fibers carrying out the maximum
ner inside the trochlea. These studies confirmed that excursion (Figure 45).
Figure 44
A Exenterated orbit specimen containing the superior B The trochlear complex including: 1) distal muscle,
oblique complex 2) trochlea, and 3) proximal tendon. These were
dissected from exenteration specimen.
* Helveston EM, Merriam WW, Ellis FD, et. al. The trochlea: a study of the anatomy and physiology, Ophthalmology,
89(2):124, 1982.
42
Surgical anatomy
A B
Figure 45
A In downgaze the direction of movement of superior C In upgaze the direction of movement of superior oblique
oblique tendon fibers tendon fibers
B Location and distance of movement of the superior
oblique insertion in: 1) downgaze, 2) primary position,
and 3) upgaze
43
Chapter 2
Reasons why the trochlea remained unde- by study of autopsy specimens demonstrated that the
scribed and undisturbed during the course of strabis- bulk of the trochlea is made up of a cartilage saddle
mus surgery for so long include its location just inside 5.5 mm long, 4 mm thick, and 4 mm wide with a
the superior orbital rim and its close relationship to groove facing the orbital wall and with a curve con-
the superior orbital vessels and nerves (Figure 46). vexed toward the bone (Figure 47). Scanning elec-
The superior oblique tendon transfer procedure, as tron microscopy demonstrates the following trochlear
first described, did include subperiosteal dislocation components (Figure 48):
of the trochlea through a skin incision, but there is no 1) Cartilage saddle
evidence that the trochlea itself was seen when this 2) A bursa-like space on the bearing surface
procedure was done. Most anatomic drawings rep- between the tendon and the groove in the
resent the trochlea in a stylized fashion, portraying it cartilaginous saddle
as a sling through which the tendon passes freely or 3) A fibrillar-vascular structure surrounding
else it is shown as a lump with the tendon entering the superior oblique tendon
on one side and exiting on the other. 4) The superior oblique tendon
Study of the trochlea first made possible by the 5) Fibrous bands attaching the trochlea to the
exenteration specimen shown in Figure 44 and later bone of the orbit
A B
Figure 46
A The trochlea attached to the medial orbital wall with the B With fascial tissues removed the superior oblique tendon
tendon entering and exiting. seen exiting the trochlea through a cuff attached to the
trochlea.
A B
44
Surgical anatomy
Figure 48
1) cartilage saddle, 2) bursa, 3) fibrillar - vascular space, 4) tendon, 5) attachment to orbital rim
The superior oblique tendon in the trochlea is Craig Gosling of the medical illustration department
made up of approximately 270 bundles of fibers. In at the Indiana University School of Medicine demon-
the several specimens studied, individual fibers in the strates the proposed dynamic relationship of the com-
bundles range in size from 0.01 to 0.1 mm. The fibers ponents of the trochlea. Significantly, the superior
appear discreet and flattened or triangular (Figure oblique tendon fibers appear to slide by each other
49). with a definite limit for each fiber, meaning that the
A description of the conclusions of the work more central fibers move farther than the more
describing the form and function of the trochlea is peripheral fibers and that the tendon moves with a
depicted in a composite drawn schematically by cumulative effect (Figure 50).
Figure 49
Scanning electron microscopy view of superior oblique tendon x320
45
Chapter 2
Figure 50 Composite
In the combined experience of strabismus sur- When these patients were studied retrospective-
geons, the superior oblique muscle has been found to ly, they had, in addition to their apparent superior
be anomalous more frequently than any other oblique palsy, a higher incidence of amblyopia and/or
extraocular muscle. The insertion varies widely in its horizontal strabismus compared to patients with
location. More importantly, absence of the superior superior oblique palsy in whom a superior oblique
oblique tendon in cases of superior oblique palsy tendon was found. Only 1 of 28 patients with congen-
has been observed and reported. In most of these ital superior oblique palsy who had a superior oblique
cases, a diagnosis of unilateral or bilateral superior tendon at surgery had amblyopia and/or horizontal
oblique palsy was made and surgery was undertaken strabismus. Also, all patients who eventually had
with the intention of doing a tuck of the superior absence of one or both superior oblique muscles had
oblique tendon. If a careful search at the insertion pronounced underaction of the superior oblique mus-
reveals no superior oblique tendon in these cases, the cle on the involved side preoperatively. Absence of
incision should be enlarged, the superior rectus the trochlea and superior oblique muscle has been
detached, and the sclera inspected from the superior demonstrated on CT (Figure 52). Patients with a
border of the horizontal recti to several millimeters diagnosis of congenital superior oblique palsy, with
posterior to the equator, including the entire anterior- or without a superior oblique tendon, have in com-
superior globe. If no superior oblique tendon is mon a superior oblique traction test suggesting a lax
found, an inferior oblique myectomy is carried out. or absent tendon and are likely to have facial asym-
Also performed is a recession of the yoke of the metry, with the larger face on the side of the paretic or
absent superior oblique, the contra lateral inferior rec- absent superior oblique.
tus, and/or recession of the ipsilateral superior rectus
(Figure 51).
46
Surgical anatomy
Figure 51
A Gaze positions showing overaction of the right inferior B At surgery, absence of the right superior oblique tendon
oblique and underaction of the right superior oblique. was confirmed.
Figure 52
A CT scan showing trochlea on the left and no trochlea on B Same patient demonstrating the superior oblique muscle
the right. on the right and no muscle on the left.
47
Chapter 2
48
Surgical anatomy
Figure 54
Schematic of the blood supply of the anterior segment from Saunders, et. al.
ACA = anterior ciliary artery IMC = intramuscular circle
LPCA = long posterior ciliary artery RCA = recurrent choroidal artery
From Saunders RA, et al. Anterior segment ischemia after strabismus surgery. Survey of Ophthalmology, 1994, 38(5):456-
466. Used with permission.
A B
Figure 55
A Normal iris filling after preoperative intravenous injection B First postoperative day after detachment and transfer of
of fluorescein in a 30-year-old man. the superior and inferior rectus muscles. Note superior
and inferior sector filling delay.
continued.
49
Chapter 2
50
Surgical anatomy
Vortex veins
There are normally four vortex veins in each eye. are observed in almost every case of inferior rectus
They are located roughly equidistant in the quadrants surgery. This occurs because the inferior vortex veins
of the globe; that is, 90 degrees apart (Figure 57). are situated about 1 mm closer to the midline near the
These veins drain blood from the iris, ciliary body, equator. Dissection of the inferior rectus is usually
and choroid. Their appearance is variable and rarely carried posteriorly to a point often posterior to the
will they number greater than four. These veins have inferior vortex veins to limit the lid effects of
a tortuous 5-7 mm intrascleral course and a similar Lockwoods ligament. The inferior temporal vortex
extrascleral course before passing through posterior vein is encountered in nearly every case when engag-
Tenons into the intraconal space. The superior vor- ing the inferior oblique in the inferior temporal quad-
tex veins empty into the superior orbital vein, and the rant. It is rare to encounter a vortex vein during sur-
remaining vortex veins empty into the inferior orbital gery on the superior or lateral rectus.
vein. If the surgeon exercises reasonable care, the vor-
In the course of strabismus surgery, each of the tex veins will remain intact. They look vulnerable but
vortex veins seems to have its own personality are actually fairly resistant to careful manipulation.
(Figure 58). The superior temporal vortex vein is Rupture of a vortex vein is rare, if surgery is done
seen at the posterior insertion of the superior oblique carefully. If a vortex vein is ruptured, it is treated
tendon. This is a reliable finding. The superior nasal with compression, and if necessary, cautery. A great
vortex vein and/or the inferior nasal vortex vein may deal of discoloration and swelling will occur, but
be seen while recessing the medial rectus, but rarely. there is no lasting complications to the surgery.
The inferior nasal and inferior temporal vortex veins
A B
Figure 57 The four vortex veins are viewed from the posterior aspect of the globe.
A Lateral B Medial
A B
Figure 58
A The superior temporal vortex vein is seen at the posterior B A vortex vein may be seen but rarely at either (or both)
insertion of the superior oblique. Vortex veins are not borders of the medial rectus.
seen routinely during surgery on the superior rectus. continued.
51
Chapter 2
C D
52
Surgical anatomy
A B
Figure 60
A Normal T1 weighted coronal MRI B Abnormal T1 weighted coronal MRI showing enlarged
inferior recti (dark area).
A B
Figure 61
A Normal T2 weighted axial MRI C Normal T1 weighted saggital MRI
B T1 weighted axial MRI with diminished signal from fat.
Note the enlarged muscle bellies.
53
Chapter 2
While coronal and saggital views are effective the case of strabismus surgery, with a smaller eye. In
for finding muscle belly enlargement, the axial view clinical practice, the most important advantages of
is best for identifying a slipped or lost muscle. using these measurements seem to be consistency and
Because of the anatomic characteristics of the rectus the ability to do the largest recession without crip-
muscles, the medial rectus is the only muscle likely to pling the medial rectus by placing the new insertion
undergo sufficient slippage to present a clinical chal- too far posteriorly.
lenge in finding the muscle. Studies of the globe in neonates and infants
Recent description of the muscle pulleys, partic- indicate that the posterior aspect of the globe is rela-
ularly those related to the horizontal recti, has tively hypodeveloped compared to the anterior
prompted Demer and associates to stress the value of aspect. This means that recession of the medial rectus
imaging for the diagnosis and treatment planning of a in a newborn could put the new insertion site behind
variety of strabismus entities. These include: the equator, even in cases where as little as 3 mm of
incomitant strabismus, A and V patterns, Brown recession were done. Therefore, it has been advocat-
syndrome, heavy eye in high myopia, and others. ed that surgery should not be done on very young
The strabismus surgeon now employs imaging infants. Surgery on infants as young as two or three
on a selective basis. Some deterrents to routine use of months has been reported, but is not something that
imaging include: high cost, inability to use in young could be considered routine or advisable based on
children, lack of need in many cases, and, of course, anatomic studies. In addition, there is good evidence
habit. It is likely, in the future, that techniques will that four months may be the earliest age that congen-
improve and costs will come down making imaging a ital esotropia can be diagnosed with confidence.
more frequently-utilized tool for strabismus manage- Surgery on infants with infantile esotropia between
ment. Ultrasound in the A or B scan mode can be the fourth and sixth month is now common and is
employed as an alternative method for orbital and safe. The axial length of the typical eye at this age is
extraocular muscle imaging. This technique is office 19.5 mm. Such eyes are entirely suitable for surgery
based and less expensive but is more difficult to inter- consisting of bimedial rectus recession to a point
pret. approximately 9.5 mm from the limbus. A safe lower
age limit for surgery in cases of congenital infantile
Growth of eye from birth esotropia with no other contraindications is four
through childhood months. Surgery for congenital esotropia by six
The eye undergoes significant growth between months of age is now common and is shown in Figure
the neonatal period and adulthood (Figure 62). Study 63.
of this growth in vivo is made possible by the use of Nanophthalmos describes an otherwise normal-
accurate, quick, and reliable A-scan biometry. The A- ly functioning eye but with a shorter anteroposterior
scan biometer, used principally for intraocular lens diameter. These eyes have increased scleral thickness
calculations in adults, has been applied to children to but decreased rigidity and are subject to retinal
obtain measurements of the anteroposterior diameter. detachment. These eyes also have hyperopia and an
Gilles first used these measurements which he com- increased incidence of glaucoma.
bined with corneal diameter measurements and meas- High myopia results in a significant increase in
urements of the medial rectus insertion site to arrive the anteroposterior diameter. Anteroposterior diame-
at a more scientific formula for recession of the medi- ters as long as 27 mm in a six-year-old child with
al recti. Kushner found an inverse relationship -11.00 D of myopia have been measured and some
between axial length and response (prism diopter adults have axial length measurements greater than
change per millimeter of surgery) in esotropic 30 mm. This can lead to intermuscular membrane
patients. This finding is expected since the maximum rupture, pulley displacement, and muscle slip causing
torque can be obtained with a smaller gear or, in eso - hypotropia or heavy eye.
54
Surgical anatomy
Figure 62
A One-week-old child, anterior-posterior diameter 17+ mm D One-year-old child, anterior-posterior diameter 20+ mm
B Three-month-old child, anterior-posterior diameter 18+ E Two-year-old child,anterior-posterior diameter 21+ mm.
mm F Three-year-old child, anterior-posterior diameter 22+ mm
C Four-month-old child, anterior-posterior diameter 19.5+ G Five-year-old child, anterior-posterior diameter 23+ mm
mm
55
Chapter 2
Sclera
The thickness of the sclera varies according to
A location (Figure 64).
1. At the limbus, the sclera is 0.8 mm thick.
2. Anterior to the rectus muscle insertions, it is
0.6 mm thick.
3. Posterior to the rectus muscle insertions, it is
0.3 mm thick.
4. At the equator, it is 0.5 to 0.8 mm thick.
5. At the posterior pole, it is greater than 1 mm
thick. The area of greatest surgical activity
for the extraocular muscle surgeon coincides
with the thinnest area of the sclera.
Care must be exercised when placing a needle
into the sclera (Figure 65). A reverse cutting needle
should be used only while exercising extreme caution
because such a needle may be as thick as or thicker
B than the sclera into which it is inserted. This could
lead to scleral perforation, an event that undoubtedly
occurs more often than is suspected or reported.
Fortunately, most cases of inadvertent scleral perfora-
tion heal without incident. If such a cutting needle is
used, it should be very fine (preferably less than 0.3
mm), if possible, and it should be inserted carefully
with the top of the needle seen through the superficial
sclera at all times. For added safety, the cutting edge
can be directed sideways so that it cuts along the scle-
ral lamellae rather than into the eye, as shown. A
curved cutting needle is less likely to perforate the
sclera than a reverse cutting needle, but the curved
cutting needle is prone to cut itself out of the sclera
unless an excessively deep bite is taken.
C A much safer needle to use is the spatula design.
With such a needle, only the tip and sides are cutting
edges. The sclera is displaced upward and downward
away from the body of the needle and is cut laterally
and ahead of the needle. This action makes the com-
plication of scleral perforation less likely to occur
with spatula needles than with cutting needles. The
spatula needles widest dimension should remain par-
allel to the scleral surface. Needles with a wire diam-
eter of .203 mm are both sufficiently strong and deli-
cate enough to be inserted safely.
A keystone spatula, with cutting tip up, is safe
Figure 63 but can cut out of sclera. A keystone spatula, cutting
A Six-month-old with 50 diopters congenital esotropia tip down, produces a longer track but can also cut in
preoperatively. to the eye. A hexagonal spatula or neutral tip needle
B Same patient immediately after surgery in the operating
room. Both medial recti were recessed to a point 10 mm must be guided to stay at mid-scleral level.
from the limbus. The axial length was 19.5 mm in each The sclera is white and opaque when fully
eye. hydrated. If this tissue becomes dried out, it becomes
C Same patient at age 1 year. dark amber-colored and translucent. Re-hydration
rapidly restores the opaque whiteness of the scleral
tissue.
56
Surgical anatomy
A B
Figure 64
A The sclera varies in thickness according to location B The sclera is thinnest, 0.3 mm, posterior to the rectus
muscle insertion
A B
D E
Figure 65
A Keystone spatula, cutting tip down D Reverse cutting - tends to cut in - can be placed
B Keystone spatula, cutting tip up sideways
C Hexagonal spatula, neutral cutting tip E Curved cutting - tends to be cut out
57
58
3
Parasurgical procedures
and preparation
Overview
The instruments required for extraocular mus- surgery. In cases where the health of a patient could
cle surgery are simple and relatively few. As with any be compromised by outpatient surgery, the patient
type of surgery, however, the surgeon must have can be admitted the day before surgery. Occasionally,
available all instruments required for a particular pro- a patient requires admission on the night of surgery
cedure, and these instruments must be in good work- on an unscheduled basis because of excessive vomit-
ing order. ing, breathing difficulties, or some other complication
Anesthesia suitable for extraocular muscle sur- which may be unrelated to the surgery itself.
gery varies according to the patient's individual The advent of mandated outpatient strabismus
requirements and the surgeon's personal preference. surgery requires that the surgeon and staff, including
Children always require general anesthesia with the operating room and recovery nurses and anesthe-
endotracheal intubation or with ketamine dissociation sia staff, participate in thorough preoperative educa-
which can be used with or without endotracheal intu- tion. This includes the family and to some degree the
bation. Insufflation anesthesia, which leaves the patient, including both the understanding child and
patient's airway unguarded except by the patients the adult. The family and/or patient should be made
own response, has been replaced in most cases by to understand that all liquids and solids by mouth
safer techniques which guard the airway. The gener- must be withheld for a period of up to 8 hours before
al anesthetic agent or agents used for children or the scheduled time of surgery. The family and/or
adults are usually determined by the anesthesiologist. patient should be assisted in obtaining necessary pre-
Cooperative adults may be operated on successfully operative laboratory tests before the day of surgery.
with local anesthesia, and a few surgeons have used In the past, this has consisted of determination of
topical anesthesia for extraocular muscle surgery in hemoglobin or hematocrit, but this is now considered
carefully selected patients. unnecessary in a healthy child. Further blood testing
The lids and face around both eyes must be is rarely required. A urinalysis is not required. Most
washed and properly draped and the operative field adult patients over 55 years of age require an electro-
freed of clutter to prepare the patient for surgery. cardiogram (ECG), which is usually performed in the
Antibiotic drops or ointment, often with steroid, are holding area just before surgery. In selected cases of
used postoperatively by nearly all strabismus sur- adult patients and less often in children with a history
geons. Infection after strabismus surgery is not com- of lung or breathing difficulties, a chest x-ray study is
mon, and rarely serious, and reaction to surgery tends required. Patients taking anticoagulants should be
to be mild. However, the use of postoperative antibi- advised to consult their primary physicians regarding
otics often with added corticosteroid is still consid- a safe time to stop and then restart this medicine.
ered worthwhile by many surgeons. A patch may be Likewise, diabetics or patients with other significant
used according to the surgeon's preference, but is usu- health issues must be identified and given proper
ally not necessary. advice, usually by their primary care physician.
Surgery is done on an outpatient basis, with A patient who lives fairly close to the facility
both children and adults arriving at the hospital on the where the surgery is performed may leave from home
morning of surgery and leaving several hours after and return home on the day of surgery. In cases
59
Chapter 3
where the patient lives farther away, i.e., more than The main purpose of the immediate preoperative
two hours by car, it may be more convenient for the examination, in my opinion, is to confirm that the sur-
patient and family to stay the night before surgery in geon has accurate information so that the proper sur-
a hotel, motel, with family living locally, or in a hos- gery will be performed. If the physical status mili-
pital-based housing facility nearby. Likewise, for tates against a safe surgical experience, surgery
comfort and safety, these patients should stay near the should be canceled and rescheduled. In cases where
hospital on the night of surgery. These patients may there is a question about a child being ill before the
be examined on the day after surgery. Other patients day scheduled for surgery, parents are advised to call
needing same day adjustment of an adjustable suture a day or two ahead of surgery and to consult their
can be retained in a short stay hospital unit. A next- local doctor about the advisability of proceeding. The
day examination is an absolute requirement if an combined surgeon-anesthesiologist history and phys-
adjustable suture must be adjusted on the morning ical examination done immediately preoperatively
after surgery but it is optional in most other cases. ensures that the patient is sufficiently healthy to
From a practical standpoint, it is necessary to undergo surgery. A sample preoperative physical
stress to patients the importance of arriving at the examination is shown in Figure 1.
hospital at the appointed time in order to avoid a shut-
down of the operating room for lack of a patient. At Consent for strabismus
the same time, it is necessary to explain that because surgery
of occasional unavoidable delays, the surgery may Before strabismus surgery is begun, appropri-
not start at the scheduled time. It is essential to pro- ate informed consent must be obtained from the
vide the family additional support during the outpa- patient or from a parent or legal guardian. A standard
tient surgery process because both the patients and operative consent form is available in most surgical
their families tend to be less comfortable as outpa- facilities. A sample of this form is shown in Figure 2.
tients compared to in-patients. In addition to the standard consent form with the
usual disclaimers used for any type of surgery, it is
Physical examination necessary to advise patients undergoing strabismus
The physical examination is simple and is usu- surgery of the following complications unique to stra-
ally completed just before surgery. However, to expe- bismus surgery:
dite the flow of outpatient surgery, it may be com-
pleted in the clinic or office up to 30 days* before Diplopia. Patients should be told they might see dou-
scheduled surgery. Likewise, laboratory tests are ble at some time after surgery. This can occur as soon
valid for 30 days*. No matter when the physical as the patient opens his/her eyes after surgery.
examination takes place, the patient's temperature is Diplopia can even be considered a favorable sign in
recorded just before surgery. In addition, at this time, patients with fusion potential. In case of an incomi-
the anesthesiologist performs auscultation of the tant deviation, a patient may be able to find double
lungs and the heart and reviews the patient's current vision looking in one direction, but also may be able
and past medications and the pertinent anesthesia his- to eliminate it looking in another. Patients are told
tory. not to be alarmed if diplopia occurs. They should be
The preoperative history obtained by the sur- told that it either goes away or if not, it can be man-
geon or team includes inquiry about upper respirato- aged successfully in nearly every case.
ry or breathing difficulties, cardiac difficulties, fever,
ear infection, bleeding tendencies, and prior anesthe- Loss of vision. Loss of vision after strabismus sur-
sia difficulties (especially family history of malignant gery can occur, but it is rare! For example, this can
hyperthermia). Any medicine used, including aspirin, occur if a needle is placed too deeply, passing through
should be recorded and made known to the surgeon. the retina and producing a vitreous hemorrhage which
Drug allergies should be noted. The history continues can clear with time, or it can cause retinal detach-
with a review of systems. Physical examination ment. Although scleral-retinal perforation may occur
includes evaluation of the heart and lungs and an in as many as 1% of strabismus cases, significant
overall observation of the patient. complications from this cause are rare. Infection pro-
The eye findings that were recorded in the clin- ducing endophthalmitis can also cause loss of vision.
ic chart at the time surgery was scheduled should be Fortunately, this too is extremely rare. Anterior seg-
reviewed, and the patient's current motility should be ment ischemia causing cataract can cause variable
compared to these findings. If there is a major dis- reduction in vision.
crepancy between these findings, it may be prudent to
cancel surgery, although this action is rarely taken.
60
Parasurgical procedures and preparation
Figure 1
61
Chapter 3
Figure 2
62
Parasurgical procedures and preparation
Need for reoperation. The need for a reoperation scissors, fine forceps, muscle hooks of various sizes,
after strabismus surgery is not really a complication retractors, needle holders, a caliper, and something to
by itself. In most cases, before doing strabismus sur- provide cautery. An open flame and a probe project-
gery, the surgeon can give the patient an approximate ing from a metal ball are used in many developing
percentage figure for the need for reoperation. For countries to achieve cautery. Although a variety of
example, the surgeon can tell the patient/family that specialized instruments have been introduced over
in spite of making a 100% effort to straighten the the years, these basic instruments have remained
eyes, there is a (10%, 20%, 30%, etc.) likelihood of essentially unchanged in number and design for a
the need for reoperation. In the case of congenital hundred years or more.
esotropia, this percentage is between 5% and 20%. In In response to finer sutures and needles, better
cases where prior surgery has been performed, when understanding of anatomy, and more widespread use
the strabismus is complicated, or after injury, need for of magnification, several modifications to the basic
reoperation may be as high as 50%. A reoperation instruments for strabismus surgery have been made.
may be necessary, even in a case where everything These instruments are now available from Katena
goes exactly as planned, because a totally predictable Products, Inc. (Figure 4). These include the
response is not possible in every case. On the other Lieberman speculum in an adult and pediatric size;
hand, in cases of a slipped or lost muscle, excessive and the Helveston Barbie retractor in three sizes,
hemorrhage, fat exposure, etc., reoperation may be standard, big, and great big, used in place of the
necessary where things did not go as planned. bulkier Desmarres retractors. Also newly modified
A separate consent obtained by the anesthesiol- are three muscle hooks including the small, right
ogist can include information about the chance of angle teaser hook to be used in place of the heavier
breathing difficulties, vomiting, sore throat, or even curved Stevens hook; three sizes of the standard mus-
of heart attack or death! cle hook with a finer hook end to be used in place of
the bulkier Jameson hook; and two sizes of a sharp,
Instruments used in pointed finder hook to be used very carefully as a
strabismus surgery combination muscle hook and dissector. A curved
The complete instrument assortment for stra- caliper is a modification of an earlier instrument
bismus surgery is shown as assembled on the instru- developed by William Scott. This newer caliper is
ment stand (Figure 3). Not every instrument is used both finer at the tip which also has a marking point
in each case, but ideally, all should be available each and has a longer handle making it easier to use. A
time strabismus surgery is done. In case the complete modification of the Moody locking Castroviejo
set is not available, the minimum instruments for stra- includes a curved, heavier handle and a more durable
bismus surgery are the following: lid speculum, fine sliding lock mechanism.
Figure 3
63
Chapter 3
A D
B E
C F
Other specialized instruments for strabismus or tenotomy whose only effect was to weaken the
surgery that have not been shown in the complete sur- muscle. Simply cutting the muscle like this frequent-
gery set are used according to the individual sur- ly produced overcorrection that was difficult or
geons preference. These include various muscle impossible to reverse. As a result, the initial wave of
resection clamps; the superior oblique tendon tucker enthusiasm for strabismus surgery waned.
in several sizes; muscle hooks with a thin metallic By the second half of the nineteenth century,
shield to guard against too deep needle placement; heavy silk sutures about the size of current 4-0 or 5-0
the heavy Green muscle hook especially used by calibre were employed to control weakening and also
some surgeons who perform the cul-de-sac incision; a for muscle shortening or strengthening. These silk
hook with a double foot to aid suture placement; and sutures were often waxed at the time of surgery to
more. help ease the passage through tissue. The re-usable
The size and style of forceps depends on the needles for introducing this suture were large, made
surgeons preference. In general, two sizes include of round wire, and had an eye to thread the suture.
the heavier size with 0.5 mm teeth and the more del- Because of the bulk of these needles compared to the
icate forceps with 0.12 mm teeth, especially useful thickness (thinness) of the sclera, sutures secured the
for grasping the conjunctiva. The design of the teeth, muscle to overlying Tenon's capsule and conjunctiva
especially those found on the 0.5 mm forceps, vary in with the knots tied externally. Muscles were not
their effect on tissue grasping and tissue tearing. The anchored to the sclera as done today. These silk
Pearse forceps features a half circle cut out on each sutures eventually had to be removed to avoid infec-
arm of the forceps that have square tips. This design tion or excess reaction.
is easier on tissue. There is also a wide array of nee-
dle holders either locking or non-locking with jaws of Animal product absorbable suture
varying size and shape suitable for the needles used Absorbable, catgut suture that is actually pro-
according to the surgeons preference. duced from sheep intestine was introduced for stra-
bismus surgery around the turn of the twentieth cen-
Sutures and needles for tury. This suture was used widely until the early
strabismus surgery 1970's. Catgut suture offered a definite advantage
over silk by being absorbable. Disadvantages of
Overview, historical perspective catgut suture are that it lacks strength and uniformity.
These sutures also produce significant inflammatory
Nonabsorbable suture - early use reaction. In an attempt to remedy some of these prob-
Strabismus surgery, introduced by Dieffenbach lems, collagen suture was introduced in the 1960's.
in 1839, was done without suture. At the outset, tech- Though still an animal product absorbable suture and
niques were limited to partial or complete myotomy no stronger than catgut, collagen promised some
64
Parasurgical procedures and preparation
improvement over catgut. Collagen suture is formed A small drawback of braided synthetic
by an extrusion of homogenized, pooled beef fascia absorbable suture is a tendency for the suture to
and is 100% collagen, making it smooth, conforma- engage tissue and drag this tissue along with the
ble, and easy to handle. Collagen suture also is easy suture (Figure 5). This can lead to premature knotting
to tie, producing a secure knot. Theoretically, the and inaccurate tissue apposition. When using this
pooled fascia making up collagen suture should result synthetic suture, it is important for the surgeon to be
in reduced antigenicity and therefore reduced inflam- aware of this and to avoid tissue drag while advanc-
matory reaction making it superior to catgut. On the ing the suture. With all of the advantages offered by
contrary, reaction with collagen is similar to that seen this new suture, this small annoyance is just that,
with catgut. Mild to moderate inflammatory reaction small and manageable. Another thing to remember
occurs in about one case in five and severe suture with this synthetic absorbable suture is that it does not
granuloma occurs in about one percent. An addition-
al drawback is that when using either catgut or colla-
gen, anyone but the most expert surgeon can expect to
break at least one suture per strabismus surgical pro-
cedure!
A
Synthetic absorbable suture
Beginning in the 1970's, synthetic absorbable
suture became available for strabismus surgery. This
material is a polymer of sugar and is called polygly-
colic acid or polyglactin 910, the latter also known as
Vicryl. This uniform, braided suture is coated with a
material similar to that used for making the suture
itself. This coating is added to smooth the suture
making for easier passage through tissue. For either
recession or resection of a muscle, 6-0 suture is suf-
ficiently strong and is the choice of most surgeons.
For closure of the conjunctiva, 8-0 suture may be
used. Some surgeons prefer to use an 8-0 collagen for B
this purpose because this suture dissolves readily and
strength is not an issue for this suture application.
In addition to being both strong and uniform,
synthetic absorbable suture is less likely to cause tis-
sue reaction as seen with animal product sutures. The
incidence of tissue reaction of any kind with synthet-
ic absorbable suture is on the order of one percent or
less. These sutures retain holding properties for 14 to
21 days, a time more than sufficient to ensure secure
healing of the extraocular muscle to sclera, something
which actually takes place in a few days. The syn-
thetic absorbable suture absorbs completely in about
three months.
In a recent survey of experienced strabismus C
surgeons, the introduction of synthetic absorbable
suture was near the top in importance of all of the
innovations for strabismus management in the past
half century. Several of those surgeons considered
the introduction of this suture to be the most impor-
tant strabismus treatment advance in this period. It is
likely that the re-introduction of adjustable sutures,
another of the strabismus related top ten events of
the past half century, was made possible largely Figure 5
because of synthetic absorbable suture. This suture is A The rough braided surface of braided Vicryl can lead to
tissue grab as it passes through Tenons capsule
strong enough to allow the manipulation required at B Comparison of 1) collagen, 2) uncoated, undyed Vicryl,
adjustment while also being absorbable and minimal- 3) coated, dyed Vicryl
ly reactive. C Poor muscle scleral union due to premature knotting
65
Chapter 3
dissolve unless buried. Exposed knots become stiff muscle to sclera? To answer these questions, Coats
and can be irritating. It is sometimes necessary to cut and Paysse placed 6-0 Vicryl sutures into human bank
off the knots of exposed 8-0 sutures used to close con- sclera with the suture track at various depths and
junctiva. lengths and then measured the force necessary to pull
Nonabsorbable suture the suture free from sclera. The results showed that
suture placed in a track 1.5 mm or longer at a depth
Nonabsorbable suture such as 5-0 or 6-0 nylon
of at least 0.2 mm required in excess of 200 gm force
or Dacron which causes minimal reaction and is supe-
to cause the suture track to fail. This is a greater force
rior to silk may be used for tucking procedures car-
than can be expected in the physiologic state, indicat-
ried out on the superior oblique tendon or rectus mus-
ing that this manner of suture placement in sclera is
cles. This type of suture is also useful for rectus mus-
both safe and effective (Figure 7).
cle union as done with the Jensen procedure or more
Some surgeons prefer the crossed swords
recently that described by Foster and Buckley for the
needle placement as described by Parks (Figure 8).
enhancement of rectus muscle transfer. The posterior
With this technique, the needles are passed through
fixation suture (retro-equatorial myopexy) or faden
sclera for approximately 5.0 mm or longer. These
operation is also a place where non-absorbable
sutures are useful.
Black silk sutures size 4-0 or 5-0 are useful for
traction during surgery. They are placed temporarily
A
near the limbus to pull the eye in a given direction to
facilitate exposure. Another use of black silk traction
sutures is to pull and then anchor the eye in an exag-
gerated direction for a few days after surgery. This
maneuver is designed to oppose early postoperative
healing that could tend to negate some or all of the
weakening effects of surgery. These silk sutures are B wire diameter
usually inexpensive and have large needles which (before fabrication)
must be inserted into episclera with care, but which C
easily pass through the lids when necessary. H I
D
Needles
The anatomy of an ophthalmic needle is E F
shown in Figure 6. The relationship of needle size
and configuration and anatomy of the sclera is dis- G
cussed in Chapter 2. The choice of a needle for stra-
bismus surgery is based in part on surgeon's prefer- Figure 6
ence, availability of needle and suture combinations, A Various dimensions F Keystone - down cutting
and the unique requirements of a given procedure. B Round tip
For recession and resection, a spatula needle with a C Cutting G Hexagon - neutral tip
D Reverse cutting H Parallel
wire diameter of .203 mm is suitable. The surgeon E Keystone - up cutting tip I Cobra head
should be aware that the configuration of the tip of the
needle will influence the path of the needle while
passing through sclera. If the tip bevels downward,
the needle will tend to go deeper into and even
through sclera. A neutral tip will go where the needle
is directed and a tip beveled upward will have a ten-
dency to cut out and therefore must be continually
directed slightly downward to stay in sclera. Any of
these needle types is acceptable, but for safety, it is
necessary for the surgeon to be aware of the needle
configuration. If there is any question, the surgeon
should examine the needle tip with magnification
before the surgery is begun.
66
Parasurgical procedures and preparation
two long shallow bites do not provide any more secu- about the safety of using succinylcholine, it should
rity for muscle attachment to the globe once the not be used. As a substitute, pancuronium, or other
sutures are tied, but this technique does allow the nondepolarizing muscle relaxant, may be used.
muscle to remain securely at the point of intended In cases scheduled for general anesthesia
recession against the sclera even before the suture is where a family history of malignant hyperthermia in
tied because of friction in the suture tunnel. When uncovered, pre-treatment with dantrolene is required.
using the crossed swords technique, the first needle Study of phenylketopyruvate serum levels can be use-
is left in place until the second needle is inserted into ful in predicting susceptibility to malignant hyper-
sclera. Each needle is then advanced in turn until
both needles just clear sclera. After this, the suture on
each needle is advanced in turn. This maneuver is
carried out to avoid having the sharp side of the spat-
ula needle cut the other end of the suture being
advanced. A needle with a down directed tip makes
this maneuver easier to perform, but extra care is
required to prevent the needle from going too deep.
Perforation of sclera by a needle placed too
deeply probably occurs more frequently than is sus-
pected. Morris, et. al. in a prospective study found
one perforation in 67 patients, 100 eyes. This is like-
ly to be a reliable statistic (1%) even for experienced
surgeons. A too deep suture rarely causes a prob-
lem. Moreover, it is not usual practice for a surgeon
to dilate the pupil and look at the retina over the site
of muscle reattachment, meaning that most of these
occurrences probably go undiscovered and unreport-
ed. This may be for the best since overzealous Figure 8
attempts at treating these mostly innocuous retinal The crossed swords technique of Parks.
perforations has resulted in serious complications
including loss of the eye (Figure 9).
67
Chapter 3
thermia in a patient with a questionable family histo- gery. There is also less anesthesia hangover and less
ry. When a patient with malignant hyperthermia or a postoperative analgesic agent may be needed. The
suspicion of this condition is to be given general induction dose is 2 to 3 mg/kg. Anesthesia is main-
anesthesia, in addition to pre-treating the patient with tained with an IV drip titrated at approximately 200
dantrolene, the anesthesia machine is flushed with mg/kg/min. Low doses of nitrous oxide or halothane
oxygen for 24 hours to rid the machine of all traces of may be used to supplement anesthesia.
halothane. The preferred general anesthetic regimen When an adjustable suture is used and adjust-
for patients with malignant hyperthermia is fentanyl, ment is contemplated for the afternoon of surgery,
nitrous oxide, and a muscle relaxant. preoperative and postoperative narcotics should be
withheld, used in a limited fashion, or reversed after
General anesthesia the operative procedure using naloxone (Narcan).
Most immature patients (younger than mid- With outpatient surgery now routine and nar-
teens) require general anesthesia for extraocular mus- cotics and barbiturates withheld from children preop-
cle surgery. This anesthetic is administered through eratively, a dramatic reduction in postoperative vom-
an endotracheal tube or with a laryngeal cuff with the iting is noted. The advantage of being able to use a
anesthetic agent(s) and oxygen delivered directly to slightly reduced amount of general anesthetic agent
the lungs. The agent most commonly used for gener- after pre-medication with narcotics is outweighed by
al anesthesia is halothane (Fluothane). Other agents the fact that after a short procedure, a heavily pre-
such as fluroxene (Fluoromar); cyclopropane; medicated patient may exhibit prolonged drowsiness,
methoxyflurane (Penthrane); and a combination of not from the general anesthetic agent, but from the
nitrous oxide, barbiturate, and narcotic may be used preoperative medications.
but have no advantage over halothane. Thiopental General anesthesia allows the surgeon more
sodium (Pentothal) given intravenously or nitrous freedom in manipulation of the muscles and accurate
oxide given by mask is the most common agent used interpretation of passive ductions. Therefore, many
for induction before intubation. Versed 0.5 mg/kg surgeons prefer general anesthesia for all strabismus
may be used as a pre-anesthetic calming drug in surgery. As with general anesthesia used for any type
excitable younger children between ages 1 and 7 of surgery, patients undergoing strabismus surgery
years. It is given in a grape-flavored liquid in the out- should be monitored constantly by the anesthesiolo-
patient holding area. Open-drop ether, which had gist in order to diagnose immediately emergencies
been used for induction of anesthesia for infants, is such as arrhythmia, hypoxia, bradycardia associated
now of historic interest. Ether has a wide margin of with the oculocardiac reflex, or cardiac arrest.
safety, but postoperative vomiting is frequent. When bradycardia from the oculocardiac reflex
Preoperative medication for infants should be occurs, all tension on the muscle should stop immedi-
limited to a moderate dose of atropine given intra- ately, and the muscle should not be placed under ten-
muscularly. The dose for preoperative atropine is sion again until the heart rate returns to normal. It is
0.01 mg/kg, with a minimum of 0.1 mg and a maxi- not necessary to remove the muscle hook from behind
mum of 0.4 mg. Older children and adults having the muscle's insertion, but the surgeon must relax all
general anesthesia may require narcotics and, in some pressure on the muscle insertion. If repeated muscle
cases, barbiturates in addition to atropine. The doses stimulation causes further bradycardia, the patient
should be selected on an individual basis. should be given intravenous atropine by the anesthe-
Preoperative narcotic is associated with a high- siologist, the dose is usually the maximum 0.4 mg
er incidence of postoperative nausea and vomiting. intravenously. The use of atropine in adults to block
With the advent of outpatient surgery, pre-medication the oculocardiac reflex increases the likelihood of
is omitted except in cases where Versed is used. cardiac arrhythmia such as bigeminy. Bradycardia
Preoperative medication with droperidol 0.075 mg/kg persisting after atropine has been injected intra-
has been said to reduce postoperative vomiting from venously may be treated with a retrobulbar injection
60% to 16% when given intravenously before muscle of 1 to 3 ml of 1% or 2% lidocaine (Xylocaine).
manipulation. This medication does not prolong the Bradycardia from the oculocardiac reflex rarely dis-
patient's recovery to full alertness or the time in the rupts the normal conduct of surgery. If it does occur,
recovery room. Outpatient strabismus surgery can be it requires just a few seconds pause. With real-time,
performed safely with only versed for pre-medication accurate monitoring, including audible pulse record-
and without other prophylaxis for postoperative vom- ing, the surgeon should be able to recognize the earli-
iting. Even with this minimalist routine, postopera- est signs of bradycardia and reduce the pull on the
tive vomiting occurs rarely. muscle. This usually leads to restoration of the
A new agent for general anesthesia is Propofal patient's normal heart rate in just a few seconds.
(Diprivan), a drug which is administered intravenous- Cardiac arrest is treated with ventilation and closed
ly. Propofal has the advantage of reducing the inci- chest heart massage both begun immediately. If car-
dence of nausea and vomiting after eye muscle sur- diac contraction does not begin after several minutes,
68
Parasurgical procedures and preparation
3 to 5 ml of intravenous epinephrine 1:10,000 may be ization of the needle before injection. Ketamine anes-
given. I have no personal experience with this or thesia is used widely, usually with other appropriate
other more radical measures. agents, in developing countries for strabismus surgery
because of its wide margin of safety.
Dissociative anesthesia Anesthesiologists skilled in the use of ketamine can
Ketamine, a dissociative anesthetic, has been administer this drug effectively and apparently with
used for a variety of ophthalmic procedures, includ- few of the drawbacks listed here.
ing strabismus surgery. With ketamine, the patient
has no cognizance of pain because the drug causes a
Local anesthesia
dissociation between the painful stimulus and any Perilimbal anesthesia
awareness of the stimulus. Involuntary movements of
Either 1% or 2% lidocaine (Xylocaine), with or
all parts of the body, including the eyes, do persist
without epinephrine 1:100,000 added, provides satis-
with ketamine, and tonus of the extraocular muscles
factory local anesthesia for strabismus surgery in
remains. Therefore, the eye must be stabilized with
cooperative teenagers and adults (Figure 10). For
traction sutures and the surgeon must be constantly on
perilimbal anesthesia, between 1 and 3 ml of the
the alert for unexpected ocular movements. The per-
agent is injected into the subconjunctival space for
sistent muscle tonus also makes interpretation of pas-
360 degrees around the limbus. This is followed by 1
sive ductions less reliable. Patients under ketamine
minute of gentle massage with the fingers through a
anesthesia also secrete saliva freely, which requires
4" x 4" gauze pad over the closed eye. Surgery may
suction during the procedure. The dose of ketamine
then begin. A variety of other techniques for anes-
is titrated to effect and is often given with other anal-
thetizing the anterior part of the eye to allow safe and
gesics or sedatives.
effective eye muscle surgery including peribulbar
In older children and adults, the combination of
anesthesia can be employed. In all cases where local
prolonged drowsiness, disturbing dreams, and hallu-
or topical anesthesia is used, oxygen is provided con-
cinations is a significant drawback to using ketamine.
tinuously via nasal catheter under the drapes. With
The disadvantages of ketamine may outweigh its
oxygen used this way, care should be exercised to
advantages for strabismus surgery. However, keta-
keep open flame or red hot thermal cautery away
mine's unique properties make it an ideal agent when
from the flowing oxygen to avoid fire. In addition, an
anesthesia is required for injection of Botox in infants
intravenous line is kept open with a normal saline
and children young enough to avoid the side effects.
drip and constant ECG monitoring is maintained. An
Persistent firing of the motor end-plates, which can be
anesthesiologist may be in attendance and may use
detected by electromyography, allows accurate local-
intravenous analgesics as needed.
A B
C D
Figure 10
A A small subconjunctival bleb is raised C Up to 3 ml of anesthesia has been injected
B Injecting into the previous bleb, the limbus is ringed D Gentle massage is carried out
69
Chapter 3
70
Parasurgical procedures and preparation
A B
C D
Figure 11
A Area of skin washed D Concave head support
B Endotracheal tube firmly anchored E Fully draped operative site using disposable drapes
C Cloth drapes (cloth may also be used).
71
Chapter 3
Figure 12
1) Patient (the most important part); 2) scrub nurse; 3) anesthesiologist; 4) surgeon; 5) assistant; 6) Mayo instrument
stand; 7) back table for drapes, solutions, etc.; 8) viewable monitors; 9) anesthesia machine
72
Parasurgical procedures and preparation
Figure 13
1 endotracheal tube 6 heating blanket
2 mass spectrograph lead 7 Doppler arterial flow sensor
3 esophageal stethoscope 8 sphygmomanometer cuff
4 nasal temperature probe 9 indwelling intravenous catheter.
5 ECG leads
Figure 14 Figure 15
Console of a modern anesthesia machine Surgeons adjustable stool
73
Chapter 3
74
Parasurgical procedures and preparation
Figure 19
1 Check in 6 Postoperative holding area (could be same room as pre-
2 Laboratory, if needed operative holding)
3 Physical examination/consent holding area 7 Discharge
4 Operating room
5 Recovery room (patient could be discharged from recov-
ery room)
75
Chapter 3
76
Section 2
79
Chapter 4
be overcorrected with the same amount of surgery 2. We frequently use Fresnel prisms in our clin-
that would produce an undercorrection in a nonam- ic for patients who have fusion potential and
blyopic patient; patients with fusion potential should are bothered by diplopia from small-angle
be slightly overcorrected; patients without fusion strabismus which may be changing. These
potential should be undercorrected, and so on. Adults prisms reduce visual acuity proportional to
without fusion potential always look better with a their strength at a rate of approximately 1 line
small-angle exotropia compared to a small-angle per 10 prism diopters. In addition, they can
esotropia.* This extra bit of insight as to how a par- yellow with age, particularly when worn by a
ticular type of patient will respond to a given amount patient who smokes. In spite of these draw-
of surgery helps the surgeon combine each patient's backs, Fresnel prisms continue to be used
needs with the type and amount of surgery required. effectively to relieve diplopia, particularly in
In this way the likelihood of a good result is maxi- the patients with residual strabismus and
mized. especially in the early postoperative period.
It should be understood that all appropriate non- They can also be placed obliquely to treat
surgical treatment that would eliminate the need for small angle horizontal and vertical strabismus
surgery or enhance the results obtained from surgery that coexist. Permanent prisms are used in
including treatment for amblyopia should be carried small-angle residual comitant strabismus
out. These include such measures as the correction of with diplopia in patients when prism therapy
hyperopia in esotropic patients suspected of having is preferred to (repeat) surgery. When cared
an accommodative element to their esotropia and, for properly Fresnel prisms can be worn
when suitable, the use of prisms, anticholinesterase effectively for long periods of time.
drops, and appropriate orthoptic exercises. Botox 3. In some cases of accommodative spasm,
treatment must be considered in selected patients, but chronic atropinization is used to eliminate
in my experience this treatment is indicated in only accommodative convergence and is com-
3% of cases. A detailed discussion of nonsurgical bined with bifocals to treat the near blur. This
treatment of strabismus will not be provided in this temporary treatment is designed to break the
book. cycle of accommodative spasm.
The following specific nonsurgical procedures 4. Occluding contact lenses or high plus contact
are worth mentioning briefly: lenses can be used in one eye in cases of
1. Anticholinesterase drugs are used for treat- intractable diplopia.
ment of refractive-accommodative esotropia. 5. A simple patch may be the only way to
Echothiophate iodide (Phospholine) 0.125% relieve a patients symptoms from diplopia.
or 0.06% used as often as one drop once each Acquired third nerve palsy is the most com-
day in each eye or as little as once per week mon and troublesome cause of intractable
in each eye can reduce or, in some cases, diplopia for adults in our practice.
eliminate esotropia. I use this treatment pri- 6. Pyridostigmine (Mestinon) for the treatment
marily as a diagnostic tool. With moderate- of diplopia from ocular myasthenia gravis is
to small-angle residual deviations in a patient commonly mentioned. However, we have
with fusion potential, anticholinesterase treat- not found that ocular myasthenia responds
ment can be used therapeutically but never well to anticholinesterase treatment.
for an indefinite period. The prolonged use Prednisone by mouth in doses from 10 to 50
of echothiophate iodide can produce iris mg or more every other day in pulsed therapy
cysts. The concurrent use of phenylephrine during episodes of diplopia may be the only
hydrochloride (Neo-Synephrine) 2.5% drops way to maintain comfortable single binocular
once each day reduces the likelihood that vision in a patient with diplopia from the
these cysts will develop. When we use anti- effects of ocular myasthenia.
cholinesterase treatment it is usually for a 7. Therapeutic orthoptics include measures such
specific, predetermined period of weeks or as diplopia awareness, fusional range
months. A common use for anti- enhancement, near point of convergence
cholinesterase treatment is in lieu of glasses training, and supervision of amblyopia treat-
when a child removes them for swimming or ment. The last two are the most commonly
while engaged in similar activities. Another used of the orthoptic treatment modalities.
effective anticholinesterase is disopropyl flu- 8. Therapeutic occlusion for amblyopia is the
orophosphate (Fluropryl) which is supplied in most commonly used nonsurgical adjunctive
ointment form. strabismus therapy. Atropine drops are also
* Look at large paintings of faces on an advertising billboard. The eyes, in order to look aligned, are painted with a large positive angle
kappa making them slightly exotropic. You will also see this in post-Renaissance portraits. Earlier artwork depicts subjects who appear
esotropic or with a reduced pupillary distance.
80
Workup of the strabismus patient
used in one eye to treat amblyopia by creating obtain help if necessary. This reassurance alone will
a blur. allay the patients concerns in most cases. It has been
9. The prism adaptation test is a nonsurgical our practice to include 6 months to 1 year of follow-
technique that helps to predict the response to up in the surgical fee. This makes follow up easier on
surgery in a patient with residual esotropia. the patient and the surgeon! Based on each patient's
To do this test, fully correcting Fresnel prisms situation, we attempt to schedule follow-up without
are placed on the glasses of a strabismus causing financial or other hardship and we individu-
patient with residual refractive esotropia who alize follow-up according to special patient needs.
is also wearing full hyperopic correction. A successfully treated congenital esotropia
Two responses are possible. In one the angle patient who also develops postoperative amblyopia
remains fully corrected with prisms after pro- requires careful follow up to monitor the amblyopia
longed wear and the patient either does or and supervise patching. If the eyes are successfully
does not demonstrate fusion with the Worth straightened, the marker for amblyopia - strabismus -
four-light. These patients have surgery for is gone, making it difficult for the family to detect
the angle measured which is equal to the persistence or recurrence of amblyopia. It is the sur-
amount of prism used. In the other response, geon's responsibility to carry out the visual examina-
the angle of deviation increases so that it tion and supervise the appropriate amblyopia treat-
measures essentially the same or close to it ment in these cases. The incidence of amblyopia in
while wearing the prisms compared to before untreated congenital esotropia is 6% according to
the prisms were placed after an adaptation Calcutt and Murray compared to between 30% and
period of hours to weeks. This response is 50% in large series of operated congenital esotropia
termed eating up the prism or anomalous patients. These data underscore the strabismus sur-
motor response. In this case, additional geon's responsibility for close postoperative follow-
prisms are placed until there is no more up of visual acuity.
response or when up to 60 are placed.
Surgery in this case is performed for the max- Step 1: Patient evaluation
imum angle found; that is, prism plus meas- The initial workup may be recorded on a
ured angle. In cases where the prisms correct preprinted sheet similar to the one illustrated in
the deviation fully in the distance but an Figure 1. The following questions should be
esodeviation remains at near, surgery is done answered and the indicated tests performed and
for the near deviation. This technique was recorded during the process of patient evaluation
subjected to a national collaborative study before strabismus surgery.
which demonstrated a small but statistically
significant improvement in results using History:
information from the prism adaptation test. Why was the patient brought in (why did he or
she come in) for an examination?
Step 4 What have the parents (what has the patient)
The fourth factor in the treatment of strabismus noted about the eyes? ET, XT, hyper, con-
is the surgical technique itself. The care and skill stant, intermittent, closes one eye, tilts head,
with which surgery should be performed will be elevates or depresses the chin, eyes jiggle,
referred to throughout the pages of this book. etc.?
Age of onset - when did the problem begin?
Step 5 Current age
The fifth component of the surgery experience is Birth weight (premature?)
follow-up. The patient's response to surgery should Growth and development
be monitored carefully in the postoperative period. o Present weight
Patching therapy for amblyopia may need to be initi- o Sat up when?*
ated or resumed, anticholinesterase drops may be o Walked when?*
* of interest primarily in infants and young
required, or prisms may be needed. In addition, com-
children
plications of surgery are always a possibility and, if
Any allergies, what medications are currently
present, should be diagnosed and treated promptly. If
being used
there is a complication associated with the surgery or
Significant illness and surgical history
if the result is less than expected - disappointing, you,
the surgeon should acknowledge this. Tell the patient
that you recognize this and be positive, but have
empathy. Let the patient know that you will see the
problem through to either remedy the problem or
81
Chapter 4
Figure 1
A preprinted examination data collection sheet is a useful adjunct to the examination, particularly for the patients first visit.
This form has been effective in our clinic, but in most instances a practitioner will design a form to meet his or her unique
needs. The importance of showing this form is that it includes most of the tests required for a complete examination.
Results of other tests such as passive duction evaluation, tensilon test, exophthalmometry, etc. may be noted on\ this form
in the appropriate area with explanatory labels.
82
Workup of the strabismus patient
83
Chapter 4
al acuity testing. In an office or clinic setting, com- acuity test or with the Worth four-lights. In this case,
puter generated visual acuity testing provides all of the word fusion is recorded. In the presence of stra-
the visual acuity testing options while at the same bismus, if one eye is preferred for fixation and the
time providing standardized illumination, random other eye deviates, the notation is fixation OD or fix-
display, and more. This instrumentation is useful but ation OS. If either eye is used for fixation with free
expensive. Relative visual acuity is alternation between the eyes or cross fixation is noted
more useful clinically than absolute acuity in an (the left eye fixes in right gaze and the right eye fixes
infant suspected of having amblyopia. Recognition in left gaze) free alternation may be present and is
acuity with Snellen optotypes (E's, the alphabet, or recorded as such. A patient may prefer one eye but
symbols) is a more stringent and, therefore, more hold fixation briefly with the non-preferred eye after
accurate test than resolution acuity done with stripes. the cover has been removed from the preferred eye.
For this reason, we test vision with Snellen optotypes Such a patient usually does not hold fixation in the
determining recognition acuity whenever we can non-preferred eye through a blink. This type of acu-
obtain cooperation from a child. ity response is recorded as prefers OD, will hold but
If decreased vision is found in each eye when not take up fixation OS, will not hold fixation through
checked monocularly, vision should be checked a blink, etc.
binocularly. The examiner in this case should be Gross, wandering fixation may be present in the
looking for latent gross or micronystagmus. Fogging non-preferred eye and should be recorded as such.
with a plus lens may be used to block vision in one Nystagmus, if present, is noted and characterized as
eye but avoid nystagmus when determining monocu- latent or manifest according to when the nystagmus
lar visual acuity in a patient with latent nystagmus. is present, and horizontal, rotary, vertical, pendular,
Near vision should be checked with E's, isolated let- jerk, and the like according to the pattern of nystag-
ters or numbers, or sentence reading, depending on mus. Frequency, amplitude, positions of greater
age. intensity, and null point are also described.
Visual acuity testing with neutral density filters Nystagmoid movements differ from nystagmus in
can differentiate functional from organic amblyopia. that the former are non-rhythmic and usually result
Vision in an eye with functional amblyopia remains at from a sensory rather than a motor defect.
or near the same level when neutral filters of increas- A peculiar type of ocular motility and head pos-
ing density are introduced. Vision in a normal eye or ture anomaly occurs with ocular motor apraxia. With
in an eye with an organic cause for poor vision this condition the eyes do not move in response to
decreases proportionally with the increased density of voluntary attempts at binocular vision through ver-
the filter. sions. Instead, the head moves past the point of fixa-
Near point of accommodation is determined in tion on the object of regard bringing the eyes along,
cooperative patients by moving a card with small so to speak. Then when the eyes have established fix-
print closer to the patient until the blur point is ation on the intended object, the head rotates back
reached. The near point of accommodation is record- while the eyes maintain fixation on the object. This
ed in diopters or centimeters. enigmatic supranuclear congenital condition tends to
Pupillary response to light is evaluated by mov- improve with age and requires no specific treatment.
ing a light from in front of one eye to in front of the Asymmetric, often unilateral, horizontal nystag-
other in reduced ambient light. A Marcus Gunn affer- mus with head nodding and torticollis in an otherwise
ent pupillary defect indicating decreased optic nerve normal child approximately 1 or 2 years old may be
function can be demonstrated with this test by noting spasmus nutans. This is a benign condition that also
a dilated pupil with the light shining in the involved requires no treatment. Spasmus nutans always
eye (direct response) and constriction of the pupil improves. Unless this condition can be clearly differ-
when the light shines in the fellow eye (consensual entiated from potentially serious causes of acquired
response). With the swinging flashlight afferent nystagmus such as chiasmal glioma or posterior fossa
pupillary defect test, as the light moves rapidly back tumor, computed tomography (CT) or magnetic reso-
and forth, both eyes dilate when the light is shining in nance imaging (MRI) of the head should be per-
the abnormal eye and both eyes constrict when the formed. There are widely differing points of view
light is shining in normal eye. regarding imaging in cases of apparent spasmus
nutans. Some pediatric ophthalmologists and neuro-
Preliminary evaluation of binocular ophthalmologists advocate imaging in every case
function while others, including myself, will do imaging only
in cases where an additional physical sign is present
Fixation such as fussiness, weight loss, or some other added
If neither eye is preferred for fixation and if the sign.
eyes appear grossly straight, fusion may be present or Vertical nystagmus with retraction is a sign of a
at least apparent. This can be confirmed with a stereo lesion around the chiasm. Patients with retraction
84
Workup of the strabismus patient
Head posture
Any face turn, chin elevation or depression, or
head tilt is noted and recorded. This observation is an
especially helpful clue in patients with vertical mus-
cle palsies and strabismus with limitation of ductions
where fusion is present. An anomalous head posture
is also a prominent feature of nystagmus with null
point. A bizarre head posture may be assumed to aid
in fusion or even in some cases to increase the amount
of diplopia to aid suppression. A simple diagram
showing the head posture and direction of gaze can be
useful. At this point any facial asymmetry, a common
finding in congenital superior oblique palsy, clefting,
skin tags, hypertelorism, unusual palpebral fissure
characteristics, etc. should be noted.
Screen comitance
Versions, or binocular eye movements, are eval-
uated in the extreme diagnostic positions comparing Figure 2
movements in the extremes of gaze carried out by Dolls head, oculocephalic response to elicit lateroversions
yoked muscles. Arrows and hash marks on the record (abduction), in an infant.
indicate overaction or underaction of the muscles.
Arrows outside the figure indicate overaction and
hash marks on the lines denote underaction. The lines
drawn as paired H figures represent the field of action
rather than the location of the yoked extraocular mus-
cles.
85
Chapter 4
First-degree fusion
The objective angle is determined after dissocia- refused at convergence of 28D. The presence of sec-
tion with the haploscope. It represents the patient's ond-degree fusion indicates that a functional result
manifest or latent strabismus (total fusion free devia- with fusion and fusional amplitudes should be obtain-
tion). Since the two targets are presented alternately, able with proper surgery. Such patients even when
this is essentially the same angle found with alternate slightly overcorrected by surgery are those best able
prism and cover testing. The subjective angle is the to obtain excellent long-term results. Fusional ampli-
angle at which the patient superimposes images of tudes can be measured in free space using the princi-
objects by manipulating the arms of the amblyoscope. ples of the haploscope but shifting images seen by the
These angles are determined clinically using dissimi- two eyes with a prism bar containing horizontal
lar, incomplete, grade I, simultaneous macular per- prisms of gradually increasing strength from 1 to
ception slides in the arms of the major amblyoscope 40D.
or other haploscopic devices; for example, a lion in a Stereo acuity. This has been called third-degree
cage, etc. Comparison of these two angles indicates fusion, but it should be recognized that these degrees
the status of retinal correspondence, at least at the of fusion are not a continuum but actually test differ-
level of dissociation created by the amblyoscope. ent things: first degree - retinal correspondence; sec-
When the objective and subjective angles are the ond degree - motor fusion; third degree - sensory
same, retinal correspondence is normal. When the fusion. The Titmus vectograph is used to test stereo
subjective angle is zero and the objective angle is acuity. Findings with this test are recorded as fly
either plus (base out esodeviation) or minus (base in (3000 seconds arc disparity); A, B, C animals; and the
exodeviation), harmonious anomalous retinal corre- fraction of the nine dots that the patient can appreci-
spondence is present. When the subjective angle is ate. The ninth dot on the Titmus vectograph describes
less than the objective angle but other than zero, non- 40 seconds of arc disparity.*
harmonious anomalous retinal correspondence is Stereopsis is not recordable in manifest strabis-
present. If no subjective angle can be determined mus of sufficient size to warrant surgery. However, it
with grade I slides, first-degree fusion is absent. may be quite good in intermittent deviations such as
First-degree fusion and normal retinal correspon- intermittent exotropia even with large angles or in
dence are favorable but by no means certain indica- small angle manifest strabismus with peripheral
tions that a functional result with fusion may be fusion.
obtained from surgery. Stereo acuity is tested using the polarized vecto-
Second-degree fusion graph method that measures the ability to fuse later-
ally displaced objects within Panums fusional space
Range of fusion. If a subjective angle is found
producing the illusion of depth. But a drawback of
with appropriate slides, grade II fusion targets are
this test is that careful study of these images can offer
inserted into the arms of the major amblyoscope and
monocular clues. Random dot vectographs have
the patient's fusional amplitudes are determined.
embedded disparity not seen monocularly. This so-
Grade II fusion slides are similar in their overall out-
called global stereopsis is said to provide more accu-
line and differ only in detail. These differences serve
rate findings of stereo acuity. Both of these tests
as checkpoints ensuring that both eyes are seeing a
require viewing with polarized glasses. Stereo acuity
target. With grade II targets in the amblyoscope, the
can also be measured without the use of spectacles
arms are first shifted from the subjective angle out-
using the Lang test or the Frisbie test. Stereo acuity
ward (exo) and then inward (eso). Fusional ampli-
can also be measured in free space using the Howard-
tudes are an expression of the patient's ability to keep
Dolman apparatus which requires the examinee to
the images as one and, therefore, fused by either
align distant objects. This test is used primarily in
diverging or converging the eyes as the arms of the
clinical research settings.
amblyoscope are shifted outward and inward.
Worth four-dot testing. Worth four-dot testing is
Fusional divergence is usually tested before fusional
performed at variable near distances and at 20 feet.
convergence. A make and break point for each is
Results of this testing are recorded as fusion, diplop-
recorded; for example, -6 to -4 and +40 to +28. This
ia, alternation, or suppression of one eye. In many
means that the patient experienced diplopia when the
instances, patients with small-angle esotropia, central
arms got to 6D exodeviation but was able to refuse
suppression, and peripheral fusion will fuse a four-
the images as the arms were returned to 4D exodevi-
light pattern that produces a large retinal image but
ation; fusion was held to 40D of convergence before
will suppress one set of lights when the retinal image
diplopia appeared and the doubled images were
is made smaller either by reducing the size of the tar-
*Titmus vectograph findings in seconds of arc disparity are: fly = 3000, cat = 400, rabbit = 200, monkey = 100, 1/9 = 800, 2/9 = 400, 3/9
= 200, 4/9 = 140, 5/9 = 100, 6/9 = 80, 7/9 = 60, 8/9 = 50, 9/9 = 40.
86
Workup of the strabismus patient
get or increasing the viewing distance. A gross esti- the lines do not intersect at the fixation point, deep
mation of the size of the central functional scotoma anomalous correspondence is present.
present during binocular vision in patients with stra- Sensory fixation. Fixation behavior is deter-
bismus can be made by determining how far the four mined with an ophthalmoscope that contains a fixa-
lights must be removed from the patient and, there- tion target that the patient is asked to look at with
fore, how small the retinal image becomes before each eye while the fellow eye is occluded. The exam-
suppression occurs. The size of the retinal image cre- iner can compare the retinal point used to fixate this
ated by the four lights as the lights recede from the target with the anatomic location of the fovea. This
patient can be calculated, but the precise value is not point of fixation is recorded directly on the chart with
clinically important. The Worth four-dot test may a small x. Fixation with any retinal point other than
also be considered a gross color vision test and a test the fovea means that acuity in that eye will be
of retinal correspondence. If four lights in proper reduced. The farther the point of fixation is from the
alignment are seen in the presence of a manifest stra- fovea, the greater the reduction in acuity. Amblyopia
bismus, harmonious anomalous retinal correspon- with peripheral eccentric fixation suggests the
dence may be inferred. This is a gross test and not possibility of a significant overcorrection of an
one that is likely to alter decisions regarding treat- esodeviation even when moderate surgery is done.
ment, although four light fusion is considered a favor- Implications of sensory testing. Sensory testing
able finding with the prism adaptation test. is useful both preoperatively and postoperatively.
Bagolini striated glasses. Bagolini glasses are The closer to normal the preoperative sensory find-
essentially see-through, micro Maddox rods that ings, the more the surgeon should try to create surgi-
turn a point of light into a line while not disturbing cal alignment or a slight surgical overcorrection that
vision. These glasses are ordinarily placed in a trial would lead to fusion. Postoperative sensory testing is
frame with their axes at 135 OD and 45 OS (or a check on surgical results and a guide to further non-
equivalent). They are used to determine retinal cor- surgical treatment that should be pursued appropriate-
respondence in casual seeing. Nearly all strabismic ly in the case of an undercorrection or an overcorrec-
patients when viewing a point of light will see diago- tion in a potentially fusing patient.
nal lines intersecting at the light or where the light is
stimulating the retina in cases with a small central Measurement of alignment - prism
scotoma. This finding is compatible with harmonious cover testing and other methods
anomalous retinal correspondence in the strabismic
Alternate prism and cover testing is a method to
patient. Some patients will see one diagonal line cor-
measure the maximum deviation. This testing is per-
responding to the preferred eye while suppressing the
formed at distance (20 feet) and near (13 inches), with
non-preferred eye. Other patients will see a complete
and without glasses (if they are worn) while the
line corresponding to the preferred eye and an incom-
patient views an accommodative target in the primary
plete line, with a missing segment adjacent to the
position. The use of an accommodative target and the
light, with the non-preferred eye. These test results
wearing of glasses are essential because together they
ordinarily do not influence surgical planning. These
control the patient's accommodative convergence.
have been used by investigators for a variety of diag-
Prism and cover testing is also done in approximate-
nostic and therapeutic manipulations and for the
ly 30 of upgaze and downgaze while the patient
study of retinal correspondence and abnormal binoc-
wears full correction and views an accommodative
ular movement.
target in the distance. If this test is performed at near
After-image test. The afterimage test is used to
while measuring for A and V pattern, the patient
determine retinal correspondence in extreme dissoci-
should wear +3.00 D lenses over the distance correc-
ation. Anomalous retinal correspondence occurring
tion to eliminate the effects of accommodative con-
on the afterimage test indicates a deep sensory anom-
vergence, especially in downgaze.
aly. Retinal correspondence tends to be normal or
Upgaze and downgaze can be achieved by tilting
harmonious anomalous when tested with the Bagolini
the patient's head forward and backward. This
glasses, normal or anomalous with the haploscope,
maneuver uncovers an A or V pattern which is best
and normal with the after-image test, indicating that
tested while the patient fixes on a distant target. A
retinal correspondence response varies with the test-
10 difference between upgaze and downgaze is sig-
ing conditions and the test. All that can be inferred
nificant for diagnosing an A pattern and a 15 differ-
from this testing is how completely the eyes have
ence is significant for a V pattern.
adapted to the strabismus angle. To do an afterimage
Other useful variations of prism and cover test-
test, a bar of bright light with a non-illuminated cen-
ing that can be performed before or after the alternate
tral fixation point is presented horizontally to the eye
prism and cover test include the following:
used for fixation and then vertically to the other eye.
1. The cover-uncover test differentiates a
If the afterimage intersects at the fixation point, nor-
tropia from a phoria. Both are measured at the
mal retinal correspondence is present with this test. If
87
Chapter 4
same time but not differentiated with the light reflex in the non-fixing eye, approxi-
alternate prism and cover test. Movement of mately 7 degrees or 15 of deviation is pres-
the covered eye immediately after the cover ent.
is removed and while the uncovered eye 6. The Krimsky test determines the amount of
maintains fixation indicates a phoria. A prism that must be placed before the fixing
tropia is noted by first establishing the fixing eye to center the corneal light reflex in the
eye and then covering it while observing the pupil of the non-fixing eye as this eye pas-
fellow eye for movement. If the fellow eye sively moves according to Herings law (see
does not move, the patient is orthotropic. If page 105). This test is particularly useful
the eye moves to take up fixation, a tropia is when the patient has such poor vision in one
present and the direction should be noted. If eye that fixation is not taken up well with that
the eyes move inward toward the nose, an eye during prism and cover testing.
exoshift is recorded; if the eyes move outward 7. Prism and cover testing with either eye fix-
toward the ear, an esoshift is recorded. When ing in the nine diagnostic positions of gaze
the eyes appear straight and/or good stereo is performed in cases of muscle palsy, partic-
acuity has been measured, even while assum- ularly vertical muscle palsy. This is the pre-
ing a face turn, head tilt, etc., the surgeon mier measurement of alignment.
should proceed to the motor evaluation. The 8. Alternate prism and cover testing with the
patient could have an incomitant mechanical head tilted approximately 45 to the right
strabismus dealt with by the patient with a and to the left is called the Bielschowsky test.
face turn, head tilt, or both. This would have This test which is said to be positive when a
been uncovered for example in a case of vertical deviation increases with head tilt is
Brown or Duane while testing ductions. A useful for identifying isolated cyclovertical
phoria is the most important feature found muscle palsy.
with the cover-uncover test. With aligned 9. Dissociated vertical deviation (DVD) is
eyes in casual seeing, a phoria can be meas- noted when either eye drifts upward the same
ured with alternate prism and cover testing. or differing amounts when occluded and
This testing also measures the total deviation, down when the cover is removed with cover-
phoria, and tropia when these coexist. uncover testing. It is recorded as +1 (5) to
2. Lateral gaze prism and cover testing can +4 (25). Some surgeons prefer to measure
reveal the presence of lateral incomitance rather than estimate DVD. This measure-
which is especially important in exodevia- ment may be performed in a manner similar
tions and in previously operated patients. to that with the simultaneous prism and cover
3. Prism and cover testing with either eye fix- test. However, DVD may be present with a
ing helps to determine the primary and sec- coexisting true vertical deviation that is in the
ondary deviation. This test is a variation of same or opposite direction as the DVD.
the simultaneous prism and cover test. DVD is also often of different amplitude in
4. Simultaneous prism and cover testing various fields of gaze and may demonstrate
(SPC) determines the actual tropia in casual movement in only one eye! DVD may even
seeing in patients where a tropia and phoria present as pseudo-overaction of the inferior
coexist (monofixational esophoria, monofix- obliques. This is confirmed by noting a
ation syndrome, microstrabismus, or small- hyperdeviation of the occluded, abducted eye
angle tropia with peripheral fusion). The fix- during lateral gaze. With true inferior
ing eye is first identified. Then it is covered oblique overaction, and no DVD the occlud-
while a prism of appropriate size and orienta- ed abducted eye is more likely to be hypode-
tion is simultaneously placed in front of the viated. Also, a V pattern should be present
deviating eye. The amount of prism is with true inferior oblique overaction. An eye
increased or decreased until no movement with DVD that moves upward when covered
occurs in the deviating eye. The prism need- can drift well below the midline when the
ed to preempt re-fixation with the deviating cover is removed. This phenomenon has
eye is a measure of the alignment during been called a falling eye. Some eyes with
casual seeing. very poor vision will drift below the midline
5. The Hirschberg test compares the location with an accompanying vertical bobbing of
of the light reflex which is normally in the the eye. This is called the Heiman-
center of the pupil of each eye to the anatom- Bielschowsky phenomenon. An exodevia-
ic central pupillary axes. It is performed tion of one eye only is called dissociated hor-
when patient cooperation is poor. For each izontal deviation (DHD). This is in a way an
millimeter of displacement of the corneal extension of DVD. The two have been char-
88
Workup of the strabismus patient
acterized as the dissociated strabismus com- afternoon. Use of atropine is usually restricted to pre-
plex (DSC) by M. E. Wilson. school-aged children, for initial refraction, and when
10. A translucent occluder* held before one eye esotropia is present.
forces fixation with the other eye but allows When refracting a lightly pigmented patient in
observation of the occluded eye. This is an the usual office setting, a cycloplegic refraction can
excellent way to observe the deviation in be performed satisfactorily approximately 20 to 40
DVD. This testing is used effectively in tele- minutes after one or two drops of cyclopentolate
medicine (see chapter 15). (Cyclogyl) 1% have been instilled in the cul-de-sac in
11. Red lens and Maddox rod tests are useful children over 1 year. One drop of phenylephrine
subjective tests and for charting in cases of (Neo-Synephrine) 2.5% may be used in addition to
small-angle vertical and/or horizontal strabis- the cyclopentolate in patients with dark irides. In
mus with symptomatic diplopia. children under 1 year of age, cyclopentolate 0.5%
12. The double Maddox rod test is useful in the drops are used.
diagnosis and measurement of cyclodevia- In esodeviating patients the full hyperopia must
tions. be elicited. Hyperopia as low as +3.00 D should
13. The 4 base-out prism test may be used to receive a trial treatment with glasses in patients with
uncover a scotoma in the macula of one eye esotropia usually beginning after 1 year of age.
in patients with microtropia. However, I have seen several patients less than 1 year
14. A Hess, Lancaster, or Lees screen may be of age with esotropia relieved by wearing a +3.00 D
used to plot directly the deviation in a coop- correction. Echothiophate iodide (Phospholine)
erative strabismic patient. drops (0.06% to 0.125%, one drop in each eye each
15. Diplopia fields are mapped with a Goldmann morning for 3 weeks) in lieu of glasses may help to
perimeter or arc perimeter while the patient determine what effect the hyperopia (accommodative
views the moving fixation target with both effort) has on the esodeviation, but because anti-
eyes open and with the head centrally posi- cholinesterase drops only reduce the effective accom-
tioned and stabilized. The documentation modative convergence/accommodation ratio (AC/A)
provided is valuable for following recovery and do not eliminate the need for accommodation,
from an acute paresis and especially for they are not a true substitute for glasses.
medicolegal and compensation purposes. A difference in the refractive error between the
eyes is called anisometropia. Difference of as little as
Refraction +1.00 or +2.00 diopters can be amblyopiogenic and
Refraction has a vital role in the diagnosis and usually indicates the need for the full refractive dif-
treatment of strabismus. It is imperative that those ference to be prescribed. If glasses are prescribed
involved in the care of the strabismus patient under- with the hyperopia reduced, it should be reduced
stand the principles of refraction, have the skills to equally. For example, OD +2.00 +1.00 x 90, OS
perform accurate measurement of the refractive error, +6.00 +2.00 x 90, could be cut to OD +1.00 +1.00 x
and use spectacle correction or the equivalent as 90, OS +5.00 +2.00 x90. These glasses would be
required in the treatment of strabismus. If you intend effective and better tolerated.
to treat patients with strabismus but are not compe- Adult patients do not ordinarily require cyclople-
tent with refraction technique, you should stop here. gia, but in selected cases of convergence excess this
Learn how to refract and then resume your study. A may be needed. It is best to avoid use of cycloplegics
person ignorant of refraction methods is not fully in pre-presbyopic adults. Use of cycloplegics in such
qualified to treat strabismus! patients can precipitate presbyopia resulting in a very
Work up of the patient with strabismus always unhappy patient.
starts with the refraction. In heavily pigmented Fundus examination
patients a cycloplegic refraction is performed using
atropine solution 1.0% (1 drop) or atropine ointment Examination of the retina is carried out using a
1% (-inch strip) in each eye for 3 days before the day standard or small portable indirect ophthalmoscope.
of examination. When drops are used, the atropine It is a relatively simple matter to see the retina poste-
may also be applied on the day of the examination. rior to the equator in a squirming infant using the
Careful instructions are given to the parents to avoid indirect ophthalmoscope. This examination to rule
overdosage. These instructions include using no out pathologic conditions in the posterior pole is an
more medicine than prescribed and holding a finger essential part of the evaluation of every strabismus
over the punctum for 30 seconds after drops are patient. Remember, esotropia is the second most com-
instilled in one eye in the morning and the other in the mon presenting sign, after white pupil of retinoblas-
89
Chapter 4
90
Workup of the strabismus patient
patient, any mechanical restriction to abduction must normal eye in cases of superior oblique palsy with
be released before the eye could be straightened and restriction of the ipsilateral superior rectus. This is
before there could be any hope of normal or near nor- due to a Herings law response with the normal supe-
mal abduction. Whether release of the medial restric- rior oblique receiving the same innervation as its
tion alone would be sufficient or whether ipsilateral yoke, the inferior rectus, in the involved eye.
lateral rectus resection or even a muscle transfer Congenital or acquired incomitant strabismus
should be performed would depend on the results of with limitations in motility caused by muscle palsies
testing for generated muscle force. If, for example, or mechanical restrictions, or a combination of the
generated force were brisk and the angle of deviation two, is usually easy to recognize. However, an accu-
small, freeing of the restriction with or without medi- rate and correct diagnosis in such cases is absolutely
al rectus recession could be sufficient. If the angle essential before a plan for surgery can be determined.
were large and the generated force only moderate, lat- Nowhere in strabismus treatment is proper diagnosis
eral rectus resection in addition to the medial rectus more essential to proper execution of surgical skills
recession might be necessary. Finally, if generated than in these cases of incomitant strabismus. The dif-
force toward abduction were minimal or nil, it would ferential diagnosis of strabismus with restricted
be necessary to do some type of extraocular muscle motility requires analysis of saccadic movements,
transfer procedure. Whether the medial rectus were forced (passive) ductions, and muscle force genera-
recessed or weakened with Botox would depend on tion in addition to the usually performed prism and
the degree of restriction and to some extent on the cover tests.
angle of deviation as well as on the surgeon's own Evaluation of noncomitant strabismus
preference.
The example shown in Figure 3 is a model for
Except in certain cases of total sixth or third
any type of strabismus with restricted motility in one
nerve palsy and a few other examples, such as inferi-
or both eyes and in one or more fields of gaze. The
or rectus denervation as occurs in certain cases of
analysis becomes more complicated as more muscles
blowout fracture, some eye movement force remains
and fields of gaze are involved, but the principles are
in the so-called underacting muscle. This remaining
the same. The patient has a right esotropia in the pri-
force, though diminished, points out that emphasis
mary position. This is the primary deviation and
should be placed on freeing of mechanical restric-
measures 50. In levoversion the eyes are grossly
tions. In some cases this is relatively easy, such as in
parallel, with normal adduction of the right eye and
cases of longstanding strabismus with a normally
normal abduction of the left eye. No muscle weak-
contracting antagonist. On the other hand, freeing of
ness or mechanical restriction is apparent in this
the restriction in a congenital Brown syndrome to
direction of gaze. In dextroversion the right esotropia
restore normal or near normal ocular motility can be
increases markedly. Abduction of the right eye is
difficult to accomplish. In addition, freeing of
deficient, whereas adduction of the left eye is normal
restricted movements can result in a distressing over-
or could be increased. The decreased abduction in the
correction.
right eye could be the result of a paretic right lateral
Overaction rectus, a mechanical restriction associated with the
Overaction of extraocular muscles is more diffi- right medial rectus or with various muscular or fascial
cult to analyze and categorize than is underaction of structures in the right eye, or a combination of the
extraocular muscles. For this reason, we use the term two. When the patient fixes with the right, paretic
apparent overaction. There is no evidence that the eye the left esotropia is increased to 70. This is
so-called overacting muscle has more force or has a called the secondary deviation.
greater saccadic velocity. Even though the eye
appears to move farther in the usual field of action. Saccadic velocity analysis
This is seen as excess adduction or abduction in long- Observed saccadic velocity is a useful clinical
standing esotropia or exotropia. This overaction may tool for differentiating a weak muscle from a normal
be seen because the checking of muscle action is muscle, which underacts only because it is held in
relaxed or relatively ineffective. In the case of over- check by mechanical restrictions. A normal extraocu-
acting oblique muscles, the apparent increase in lar muscle will produce a brisk movement of the eye
action may be a horizontal movement which allows with a peak velocity of 400 per second to as high as
the globe to move farther laterally in the orbit. 600 per second. A paretic muscle will usually pro-
Careful observation of the apparent overacting supe- duce velocities at about one-tenth this speed. This
rior and inferior oblique frequently reveals that over- movement may be produced not by the paretic mus-
action is actually abduction. This produces the well- cle but by elastic orbital forces influenced by relax-
known V with overaction of the inferior obliques ation of the antagonist of the paretic muscle.
and A with overaction of the superior obliques. Decreased saccadic velocity can be readily observed
Overaction of the superior oblique can be seen in the in the clinical setting. The electro-oculograph pro-
91
Chapter 4
Figure 3
A Fixing with the sound eye (primary deviation) - a right C Dextroversion shows limitation of abduction in the right
esotropia is observed. eye.
B Levoversion is essentially normal. D Fixing with the paretic eye and/or mechanically restrict-
ed right eye produces a larger secondary deviation.
92
Workup of the strabismus patient
vides recordings useful for determining subtle defi- function is in question. The patient is then asked to
ciencies and for purposes of documentation in the abruptly switch fixation to an object in the field of
laboratory. But for clinical evaluation, observation action of the suspected paretic muscle. In this case
alone is usually sufficient. the patient is asked to switch fixation from extreme
Saccadic velocity analysis begins with the patient levoversion to extreme dextroversion. To accomplish
fixing on an object in the field opposite that of the this maneuver, it is best to instruct the patient to look
suspected paretic muscle (Figure 4). The patient at an object that the examiner holds in the patient's
shown has a right esotropia. To begin the test for sac- far left field and then to look at an object held in the
cadic velocity, the patient is asked to look far to the examiner's hand in the patient's far right field. During
left in the field opposite that of the muscle whose the patient's switch of fixation, the examiner observes
Normal innervation
Paresis v. paralysis
Figure 4
A Eyes are in a gaze opposite the field of action of the C A slow or floating saccade indicates decreased innerva-
underacting muscle tion of the right lateral rectus. No information is avail-
B Fixation shifts from extreme left to extreme right gaze able about possible mechanical restriction.
resulting in a saccade. In this case the eyes move with
equal velocity but excursion of the right eye is limited
suggesting normal innervation to the right lateral rectus.
Restricted right eye movement is likely due to mechani-
cal factors.
93
Chapter 4
the speed of the movement in the eye with limited Forced (passive ductions)
motility (the right eye). If this eye moves at a normal
saccadic speed (200 to 400 degrees/sec), as does the Forced or passive ductions should be carried out
normal eye which serves as a control, the apparently at some time on all patients undergoing strabismus
underacting muscle (the right lateral rectus) is proba- surgery. This test can be done at the time of surgery,
bly contracting in a normal or nearly normal way. after adequate anesthesia has been obtained. The test
The limited motility is probably caused by mechani- is performed in both eyes in all directions. In most
cal restriction associated with this muscle's antagonist cases, forced or passive ductions are performed in the
or other fascial structures around the globe. On the operating room just before the actual surgery is
other hand, if the eye moves to its final position in begun. The test is accurate immediately if a non-
attempted dextroversion with a slow, floating move- depolarizing muscle relaxant has been used during
ment ( 30 degrees/sec), which is slower than the fel- general anesthesia, but can only be performed after 15
low eye, this evidence suggests that the right lateral or 20 minutes if succinylcholine or equivalent has
rectus is paretic. In this case, little can be determined been used because of muscle contraction.
regarding the presence or absence of an associated In cooperative patients with restricted motility
mechanical restriction. A possible co-existing about whom the surgeon wants as much information
mechanical restriction is determined only after pas- as possible before going to the operating room, forced
sive duction testing has been performed. Saccadic ductions can be carried out in the office using topical
velocity analysis also can be performed with the aid anesthesia. Several drops of proparacaine hydrochlo-
of an electro-oculograph that provides a printed read- ride or tetracaine are sufficient to anesthetize the con-
out. The electro-oculograph measures peak velocity junctiva. In place of these anesthetic agents, 5% lido-
on one track. A second track measures the magnitude caine (Xylocaine) drops may be used. As another
of the ocular movement and displays a slope of the alternative for anesthesia, a cotton-tipped applicator
ocular movement speed (Figure 5). saturated with cocaine hydrochloride 4% is held
against the conjunctiva at the point where the forced
Figure 5
A Limited movement in the right eye with normal saccadic B A slow saccadic velocity indicates weakness of the right
velocity indicates that restriction is causing the strabis- lateral rectus muscle. No information is revealed about
mus. possible coexisting restrictions. This must be analyzed
with forced duction testing. Peak velocity is not shown.
94
Workup of the strabismus patient
duction forceps are to be applied. Fine-toothed for- Traction testing of the superior oblique tendon
ceps are used to grasp the conjunctiva and episclera, can reveal laxity of the tendon which is the hallmark
and the patient is asked to look toward the field of of anatomic congenital superior oblique palsy with
action of the restricted motility. The examiner then its frequent anatomic anomalies of the tendon. When
gently assists the eye into the full extent of the laxity of the superior oblique tendon is found, it is
attempted duction. much more likely that the superior oblique tendon
Three important techniques to practice when will be explored, found to be loose or abnormally
doing the forced ductions on a patient in the clinic are inserted, and subsequently tucked, resected, or redi-
1) gently lift the eye as it is rotated on the physiolog- rected. On the other hand, if the tendon is thought to
ic axis center of rotation while avoiding pushing the be normal based on the superior oblique traction test,
globe back in the orbit, 2) grasp the conjunctiva- epis- the diagnosis is more likely acquired superior oblique
clera with a secure bite with the forceps, and 3) palsy or neurogenic congenital superior oblique palsy
instruct/reassure the patient to continue looking in the and tuck or resection of the superior oblique is not
field to be tested and avoid a refixation that could performed, avoiding a postoperative Brown syn-
cause the cornea to be scraped by the forceps creating drome. This superior oblique traction test adds to the
a corneal abrasion or tearing the conjunctiva. accuracy of both diagnosis and treatment of superior
It has been suggested that passive ductions can oblique palsy. Saunders and later Plager described an
be tested using just a cotton-tipped applicator to move intraoperative traction test to determine the proper
the globe after instillation of a topical anesthetic. amount to tuck the superior oblique tendon. Guided
This technique avoids the complications just by this test the tuck can be loosened if the tendon is
described. found too tight or tightened if the tendon remains lax.
In the example shown, the patient has a right To perform the superior oblique traction test on
esotropia and limited abduction of the right eye the right eye, shown from above in Figure 7, the eye
(Figure 6). After anesthetizing the eyes the patient is is grasped at the limbus with fine-toothed forceps at
asked to look as far to the right as possible. The con- the 4 to 10 o'clock positions (shown) or the 2 and 8
junctiva and episclera of the right eye are grasped o'clock positions (not shown) on the left eye. The
with a fine-toothed forceps at the nasal limbus (3 view is from above the patient's head. The eye is
o'clock position). The examiner then attempts to pushed back in the orbit in full adduction. The eye is
abduct the right eye gently but forcibly while follow- then brought temporally while continuing to push it
ing the normal arc of rotation of the eye around its back in the orbit. A normal taut superior oblique will
physiologic vertical axis. If the eye cannot be abduct- cause the globe to pop up. This reaction can be felt
ed fully, a mechanical restriction is present and the and seen. As the globe slips over the superior oblique
limitation of abduction results from mechanical caus- tendon toward abduction, the eye recedes a bit further
es with or without associated paresis of the lateral in the orbit. If no tendon is felt or a very slack ten-
rectus (as inferred from saccadic velocity analysis). don is felt and the eye does not pop up but instead
If the eye can be abducted fully and the examiner slides back into the orbit when the globe is brought
feels no resistance to forced abduction, no mechani- temporally, the superior oblique is loose or absent.
cal restriction is present and a right lateral rectus pare- Regardless of the tightness or looseness of the tendon,
sis is indicated. This finding is always associated the eye is easily pushed back in the orbit in full
with a floating saccade. abduction. In the case of a very loose tendon the
cornea remains out of view during the temporal
Passive duction testing of the oblique excursion of the globe.
muscles Inferior oblique traction test
Superior oblique traction test The inferior oblique traction test is best used to
confirm that apparent inferior oblique overaction is
Guyton suggested an ingenious scheme for eval-
due to a taut inferior oblique muscle and is done when
uating the status of the superior oblique tendon. This
inferior oblique overaction persists after the muscle
test depends on feeling a knife edge response when
has ostensibly been weakened. When performing this
gently pushing the globe backward in the orbit while
test, it is not uncommon to find a tight inferior
rolling the globe back and forth over the stretched
oblique response in a muscle that has been previous-
superior oblique tendon. This test is interpreted
ly weakened. When this is encountered and the infe-
according to the surgeon's experience. It is performed
rior oblique is explored most likely either the cut ends
in the operating room with the patient under general
of the muscle have reunited or some other type of
anesthesia to achieve the proper level of muscle
fibrous attachment is effective in causing persistent
relaxation. Plager has expanded on the testing for
inferior oblique overaction. In either situation, sever-
tightness (or laxity) of the obliques, adding testing of
ing the attachments that cause inferior oblique func-
the inferior oblique muscle.
tion lateral to the inferior rectus is usually effective
95
Chapter 4
Figure 6
A Fixing with the sound left eye (primary deviation) D If the eye goes freely into abduction, passive or forced
B In dextroversion, limited abduction of the right eye is ductions are free confirming that no mechanical restric-
seen. tion is present.
C After topical anesthetic has been applied, the examiner
attempts to complete abduction of the right eye. If this
is not possible - the eye is stiff and immovable -
mechanical restriction is present.
96
Workup of the strabismus patient
Figure 7 The superior oblique traction test (viewed from above the patients head)
A The eye is grasped at the 2 oclock and 10 oclock posi- C With a lax or loose tendon the cornea disappears and
tion (right eye from above) in preparation for the superior remains hidden behind the upper lid as the eye is rotat-
oblique traction test. ed.
B The eye is pushed back into the orbit and is guided from D The relative path of the globe as it passes over a normal
nasal to temporal. As it goes over the normal superior tendon.
oblique tendon, the eye pops up. E A lax superior oblique tendon allows the globe to be
pushed backward into the orbit.
97
Chapter 4
provided that the case is not otherwise complicated. hydrochloride 4% held against the conjunctiva at the
In cases of inferior oblique adherence or inferior point where it is to be grasped with the forceps may
oblique inclusion, however, the outcome of reopera- also be used to anesthetize the conjunctiva. The anes-
tion is not so optimistic. Restrictions often persist in thetized conjunctiva and episclera of the right eye are
spite of careful attempts to free all adhesions in the grasped with fine-toothed forceps at the nasal limbus
infero-temporal quadrant. (3 o'clock) and the patient is asked to move the eye
To perform the inferior oblique traction test, the slowly far to the right while the examiner attempts to
eye is grasped at the limbus with fine-toothed forceps stabilize the right eye in extreme adduction. The
at the 2 and 8 o'clock positions on the right eye (or the amount of tug felt by the examiner through the for-
4 and 10 o'clock positions on the left eye), shown ceps indicates the contracting power of the right lat-
from above in Figure 8. The eye is pushed back in the eral rectus. If no appreciable tug is felt, little or no
orbit in full adduction. The eye is then brought tem- contraction of the right lateral rectus occurred. This
porally while continuing to push back in the orbit. A type of response is associated with a floating saccade.
normal or taut inferior oblique will cause the globe to If the examiner feels a brisk tug on the for-
pop up. This reaction can be felt and seen. If no ceps stabilizing the eye, the right lateral rectus has
taut muscle is felt and the eye does not pop up when significant contracting power. This type of response
the globe is brought temporally, the inferior oblique is is associated with a brisk, normal-velocity, saccadic
not tight and probably has been effectively weakened. movement. The limited movement in this case is
Regardless of the tightness or looseness of the mus- caused by a mechanical leash or tethering effect
cle, the eye is easily pushed back in the orbit in full usually caused by scar tissue, adhesions, or a spastic,
abduction. contracted antagonist. The surgeon can test generat-
ed force in the normal eye of a patient after topical
Generated muscle force anesthesia to establish a feel for this test.
Another step in analysis of strabismus with More accurate determination of active mus-
restricted motility is to perform the active muscle- cle force generation can be obtained by using a strain-
force generation test. This test determines, in the gauge. Black silk sutures (4-0 or 5-0) are affixed to
presence of restricted eye movements, the amount of the episclera and attached to the strain gauge deflec-
force generated by a given extraocular muscle within tor. A perilimbal suction cup or a special forceps can
the range of movement noted on testing of versions be used to connect the episclera to a suture or other
and ductions. Active muscle force generation is a tac- device that in turn attaches to the strain gauge.
tile test that complements saccadic velocity analysis Isometric contractions should be 60 to 90 gm or more
which is a visual test. in a normal muscle and are reduced to approximately
The information obtained from saccadic analy- 10 gm in complete paralysis. Even with paralysis
sis, forced ductions, the muscle-force generation and some force is measured because of passive tissue
in a few cases the differential intraocular pressure test forces. These more accurate tests are reserved for the
helps indicate if recession-resection and freeing of laboratory.
restrictions are indicated or if muscle transfer with or
without freeing of restrictions is required. When nor- Differential intraocular
mal contraction plus mechanical restriction is present, pressure test
the restriction first must be eliminated by freeing con- The differential intraocular pressure (IOP) test is
junctival-globe-muscle-fascial adhesions. Detaching a useful indirect technique for diagnosing generated
and recessing or otherwise weakening a muscle may muscle force and confirming mechanical restriction
be required. Either recession alone or a recession- in the face of a normal agonist. This test is based on
resection procedure should be performed as indicat- the fact that in the normal patient the eye rotates
ed. When reduced or weak muscle contraction is dis- around the center of the globe. During normal move-
covered, as evidenced by reduced generated muscle ment the antagonist relaxes as the agonist contracts
force, a muscle transfer is usually indicated. This is and no excessive pressure is placed externally on the
done with or without weakening the antagonist based globe, and IOP remains normal. At extremes of duc-
on the forced duction test, the age of the patient, and tions in the normal eye, an increase in IOP occurs
the number of undisturbed anterior ciliary arteries. because ocular movement is stopped by mechanical
To start the test, the patient is instructed to look checking by the antagonist rather than by lack of ago-
in the direction opposite from the field of action of the nist contraction power. In cases where ocular move-
muscle to be tested. In the example cited (a right ment is limited by mechanical restriction, the IOP
esotropia with limited abduction of the right eye), the increases when the eye attempts to move into the
patient is asked to look far to the left (Figure 9). restricted field. On the other hand, when a restricted
Several drops of proparacaine hydrochloride (0.5%) movement is not accompanied by an increase in IOP,
are sufficient to anesthetize the cornea and conjuncti- paresis may be indicated. The differential IOP test
va. A cotton-tipped applicator saturated with cocaine
98
Workup of the strabismus patient
A B
C1 C2
Figure 8 The inferior oblique traction test viewed from above the patients head
A The right eye is grasped at the limbus. C2 If no pop is felt, the inferior oblique has been effective-
B The eye is rotated nasally and pushed back in the orbit. ly weakened.
C1 The eye is brought temporally -- the surgeon feels for D The eye normally recedes in abduction regardless of
the pop up of the inferior oblique which is still present. the state of the inferior oblique.
99
Chapter 4
B1
a slight tug
signifies a weak
muscle
B2
Figure 9
A To test abducting force in the right lateral rectus, the B2 If the tug on the forceps is felt as strong then it confirms
right eye is treated with appropriate topical anesthesia that the muscle is innervated - not paralyzed.
and the patient is instructed to look in extreme left gaze. C If the examiner has difficulty fixing the eye nasally, the
B1 The right eye is grasped at the nasal limbus (or temporal restraining forceps can be placed temporally (9 oclock
limbus) with fine-toothed forceps. The patient is asked in the right eye). Great care must be exercised to avoid
to look slowly to far right gaze. If only a slight tug is felt losing the firm grasp of the eye that could result in
in the muscle tested (the right lateral rectus in this case), scratching the cornea. The tester should remain alert
a paresis or paralysis is confirmed. to pull the forceps away if the grip is loosening.
100
Workup of the strabismus patient
can be used in the evaluation of strabismus with ment is limited by a restriction, the point of restriction
restriction in a patient at any age, but it is especially becomes a fulcrum, the eye is pulled backward, and
useful in a patient who will not cooperate for other the IOP increases. A longstanding right esotropia
tests of generated muscle force. Patients with thyroid with normal lateral rectus function in the right eye but
ophthalmopathy especially involving the inferior rec- restriction of the antagonist right medial rectus is
tus often have chronic elevation of intraocular pres- shown. This condition can occur after recovery from
sure by this mechanism. Optic nerve changes and a sixth nerve palsy or with co-contraction in Duane.
visual field defects characteristic of glaucoma have Orbital resistance meeting the backward-pulled eye
been seen in such cases. produces an elevation in IOP, as recorded earlier in
The eye normally rotates around its center, the the primary position. If in the cases cited previously
antagonist relaxing and the agonist contracting with the limited abduction of the right eye were due to
no increase in IOP until the extremes of duction have paresis of the right lateral rectus, no rise in IOP would
been encountered (Figure 10). When an ocular move- occur on attempted abduction.
A
15 mmHg 15 mmHg
Figure 10
A As the eye rotates in adduction around the Z axis no B As the eye attempts to rotate in adduction a stiff, non-
additional pressure is exerted and intraocular pressure is relaxing lateral rectus becomes a fulcrum, the globe
unchanged. retracts and pressure rises.
continued.
101
Chapter 4
C1
C2
102
Workup of the strabismus patient
Figure 12
The superior oblique depresses, intorts and abducts. The superior rectus elevates,
adducts, and intorts.
103
Chapter 4
abducts, and intorts the globe. In addition, the anteri- When a horizontal rectus muscle is shifted verti-
or fibers of the superior oblique tendon are primarily cally, this muscle has less effect on the globe align-
responsible for intorsion and the posterior fibers for ment in the same direction as the muscle is shifted.
depression. If the superior oblique tendon is moved For example, when the medial rectus is shifted up,
forward, it will increase intorsion. If the superior rec- this muscle has less adducting effect in upgaze.
tus is moved temporally, it will increase intorsion, When the medial rectus is shifted downward, adduct-
and if it is moved nasally, it will decrease intorsion. ing effect is less in down gaze. The same applies for
Both of these superior muscles move the globe in the the lateral rectus. This response is the basis of verti-
x-, y-, and z-axes. cal shift of the horizontal recti to treat A or V pat-
The globes are shown as viewed from below in tern.
Figure 13. The inferior rectus (IR) depresses, As a rule, all previously operated muscles that
adducts, and extorts the globe. Recessing the inferior are being considered for surgery should be inspected
rectus as done in thyroid restrictive disease can result under direct visualization before any strengthening,
in decreased adduction and an A pattern. This can be weakening, or transfer procedures are performed. In
avoided by a nasal shift. The inferior oblique (IO) ele- case of reoperation, findings at the time of surgery
vates, abducts, and extorts the globe. Both of these could lead to change in the surgical plan. For exam-
inferior muscles move the globe in the x-, y-, and z- ple, in a patient with secondary exotropia occurring
axes. after recession of the medial rectus and resection of
In the primary position, the medial rectus (MR) the lateral rectus for esotropia, depending on the
and lateral rectus (LR) muscles adduct and abduct the angles, the lateral rectus would require weakening
globe, respectively (Figure 14). The eye is viewed and the medial rectus strengthening. If treated like a
from the lateral aspect. These muscles move the new case, the lateral rectus would be weakened first
globe around the z-axis. With the eye elevated, both and then the medial rectus strengthened. This
horizontal rectus muscles elevate the globe (Figure approach follows the rule of doing the recession first
15). This adds movement of the globe around the x- in a recession-resection procedure. However, in sec-
axis. With the eye depressed, both horizontal rectus ondary cases where two muscles will be operated and
muscles depress the globe. Both horizontal rectus forced ductions are unrestricted and ductions limited,
muscles can be shifted upward to improve elevation the muscle to be strengthened is isolated first and
or downward to improve depression (Figure 16). tagged with a 4-0 silk suture. This is a good practice
When the medial or lateral rectus insertion is shifted to follow because it allows the surgeon to determine
upward, the muscle becomes an elevator in part. if a previously operated muscle is indeed present or,
When the medial or lateral rectus insertion is shifted if present, has slipped from the intended point of
downward, the muscle becomes a depressor in part. insertion. The condition of the muscle to be strength-
Mechanical restrictions hampering ocular move- ened by advancement and resection may influence the
ments can be associated with the following: (1) con- amount of weakening that should be done on the
junctiva, (2) anterior Tenon's capsule, (3) the muscle antagonist, if any at all, or may indicate that a muscle
itself, (4) intermuscular membrane, and (5) orbital fat transfer should be performed. For example, esotropia
(Figure 17). All of these factors must be considered occurring after recession of the lateral rectus and
during surgery. resection of the medial rectus for exotropia and with
Figure 13
The inferior oblique elevates, extorts, and abducts. The inferior rectus depresses,
adducts, and extorts.
104
Workup of the strabismus patient
Figure 17
Mechanical restriction limiting eye movement can be association with:
1 Conjunctiva 4 Intermuscular membrane (posterior Tenons)
2 Anterior Tenons 5 Orbital fat
3 The muscle
105
Chapter 4
A B
C D
E F
G H
106
Workup of the strabismus patient
Hering's law, the yoke of the muscle responsible for test that sorts out paretic obliques and vertical recti
elevation in abduction (left superior rectus) which is (Figure 19).
the right inferior oblique will receive more innerva- During upgaze the inferior oblique muscles can
tion. By this technique the right inferior oblique can overact causing a V pattern. The abducting force of
be strengthened. If in this example the eye with the the inferior obliques producing a greater exodeviation
paretic muscle is used for fixation, weakening the in upgaze results from weakness of the superior
yoke will simply move the fellow eye down to align obliques, upward displacement of the medial pulleys
with the eye with the underacting muscle. This prin- or weakness of adduction after recession of the medi-
ciple is applied with the posterior fixation suture to al recti. Antimongoloid fissures are common in V
produce a laudable secondary deviation. esotropia (Figure 20).
In addition to the six positions of gaze testing In downgaze, overaction of the superior obliques
the action of these yoked muscles, the alignment of results in an A pattern that is seen in both exo and
the eyes is observed in primary position with the head esodeviations. Overaction of the superior obliques is
tilted 45 to the right and the left, and looking 30 up seen with mongoloid fissures and with downward dis-
and 30 down. placement of the medial pulleys.
The fact that the oblique and rectus muscles are As mentioned previously, non-surgical treatment
torsional synergists but vertical antagonists is the will not be discussed in detail in this text. All appro-
basis for the Bielschowsky head tilt test which priate nonsurgical treatment including glasses,
demonstrates greater vertical deviation when the head prisms, anticholinesterase, occlusion, orthoptics, and
is tilted toward the side of the paretic superior the like should be carried out in appropriate cases
oblique. This is also the key step in the Parks 3-step before embarking on surgery.
A Parks 1
B Parks 2
Helveston Step I In a case with a hyperdeviation, the adducted eye in the lateroversion of greater vertical deviation
points to the ipsilateral oblique and contralateral rectus as the possibly paretic muscle.
C Parks 3
Helveston Step II If the vertical deviation is greater with head tilt toward the higher eye, the oblique from Step I is paret-
ic. If the opposite is true; that is, the vertical deviation is greater with the head tilted toward the lower eye, the vertical rec-
tus from Step I is paretic.
Figure 19 Positive Bielschowksy test in a patient with right superior oblique palsy
A Right hypertropia shown here latent because of vertical right superior rectus as it responds to the need to intort the
fusional amplitudes right eye. This is shown here as the Parks Three Step
B Overaction of the right inferior oblique in levoversion Test.
C With head tilt to the right the hypertropia increases
because of the weakly opposed elevating action of the
107
Chapter 4
B C
D E
Figure 20
A Antimongoloid fissures are associated with V pattern C Looking down the abducting action of the superior
esotropia. Mongoloid fissures are associated with A oblique can produce an A.
esotropia. Esotropia in this patient is in upgaze only. D Lax superior oblique tendons, upward displacement of
This rule does not necessarily hold for exodeviations. pulleys and recessed medial rectus contribute to a V.
B Looking up the abducting action of the inferior obliques E Downward displacement of the pulleys contribute to an
can produce a V. A.
108
Workup of the strabismus patient
Individuals with esotropia, who have an angle Upward displacement of the medial pulleys or
sufficiently small to be corrected by a single medial downward displacement of the lateral pulleys or con-
rectus recession, tend to have peripheral fusion and genital laxity of the superior oblique tendons are also
harmonious anomalous retinal correspondence and a likely cause of a V pattern. In either case, the infe-
are included in the monofixation syndrome. Such rior oblique may apparently overact but the real
patients are usually cosmetically acceptable and are cause for hyperdeviation in adduction and for abduc-
often better without surgery. A single medial rectus tion in elevation (producing the V) is deficient
recession performed for fear of producing an over- checking with a lax tendon or shifted vectors with
correction is usually a manifestation of trepidation on pulley displacement.
the part of an overcautious surgeon. Single medial The desire for symmetry is not necessarily an
rectus recession for patients with limitation of motili- indication for performing a bimedial rectus recession
ty such as Duane syndrome is worthwhile and is dis- in every case of esotropia. A recession of the medial
cussed elsewhere (see chapter 5). rectus and resection of the lateral rectus is indicated
Resection of a single lateral rectus muscle for in cases with poor vision in one eye or with other rea-
esotropia is less effective than recession of a single sons to limit surgery to one eye. In addition, some
medial rectus muscle and is less likely to be indicated surgeons simply prefer to perform recession-resection
except in specific cases such as a lateral rectus mus- and this is perfectly acceptable.
cle that has slipped after prior surgery. Advancement Bimedial rectus recession measured from the
of a previously recessed lateral rectus can be effective limbus. Since 1975 I have measured all bimedial rec-
in cases of small angle incomitant esotropia with lim- tus recessions using the limbus as the reference point.
ited abduction after lateral rectus recession. This is done for two reasons. First, the medial rectus
insertion site was found to be extremely variable,
ranging from 3.0 to 6.0 mm (average 4.4 mm) in a
series of esotropic patients, with the insertion site
having no relationship to the angle of deviation.
Two muscle surgery for esotropia: Second, up to 50% of patients undergoing bimedial
rectus recession for congenital esotropia before this
Bimedial rectus recession measured from the
time (when maximum medial rectus recession was
insertion. A minimal bimedial rectus recession of 2.5
5.0 to 5.5 mm) required additional surgery because of
mm reduces an esodeviation approximately 15 to
undercorrection of the esodeviation. The unaccept-
20. A maximum bimedial rectus recession of 7
able number of undercorrections suggested that a
mm* results in as much as 40 or more reduction in
larger bimedial rectus recession should be performed
the esodeviation. Slightly more effect may be
provided it could be accomplished safely.
obtained in infants, but definitely less effect is pro-
Rationale for measuring from the limbus.
duced in adults. Indications for bimedial rectus reces-
Assuming that the medial rectus muscles could be
sion include:
recessed to the equator without crippling the action of
1. Congenital esotropia up to 50
the muscle, we decided to move the medial rectus
2. Esotropia in an adult up to 40
muscle to the equator and consider this a maximum
3. Equal vision
recession for large-angle congenital esotropia. The
4. Esotropia greater at near (high AC/A)
medial recti could then be recessed a lesser amount
5. Excess adduction
for smaller angles while still measuring from the lim-
A bimedial rectus recession with vertical shift
bus. The easiest way to locate the equator is to use
may also be performed in patients with an A or V pat-
the limbus as a reference. Since the corneal diameter
tern, especially in patients without oblique dysfunc-
defined by the limbus as well as the axial length are
tion. Lack of oblique muscle overaction is determined
fairly consistent according to patient age, it is rela-
by noting absence of hyper- or hypo-deviation in the
tively easy to calculate the distance of the equator
adducting eye in latero-version. In this case, the
from the limbus. In the infant between six months
medial rectus muscles are shifted vertically toward
and one year, this value is approximately 10.5 mm.
the closed end of the pattern. This means that the
Between four and six months the maximum is 10.0
recessed medial rectus muscles are shifted upward for
mm. In the child over one year, the equator is approx-
an A pattern and downward for a V pattern.
imately 11.5 mm from the limbus. Therefore, these
In patients with a V pattern with inferior
dimensions were utilized as guides for performing
oblique overaction, a bimedial rectus recession and
bimedial rectus recessions. These maximum reces-
bilateral inferior oblique weakening is usually the
sions have been used for any large angle of congeni-
preferred technique.
tal esotropia, even those in excess of 50. On the
* This might be excessive in a normal or smaller eye with a medial rectus insertion 5.5 mm from the limbus.
109
Chapter 4
25 8.5 mm 25 8.5 mm
35 9.5 mm 35 9.5 mm
Table 1
other hand, smaller deviations receive smaller reces- cases without tight conjunctiva, and this includes
sions with a minimum bimedial rectus recession for most cases with congenital esotropia, I use a cul-de-
congenital esotropia being 8.5 mm from the limbus. sac incision.
Intermediate deviations are titrated between these
numbers and are described above (Table 1). It should
be emphasized that these numbers are merely guide-
lines. The surgeon should individualize his/her surgi-
cal numbers according to experience.
Measuring from the limbus has proved to be an Bilateral lateral rectus resection. I seldom per-
effective way of performing a larger bimedial rectus form bilateral lateral rectus resection. It is used most-
recession without crippling the effect of the medial ly as a second procedure by surgeons who routinely
rectus muscle postoperatively. This technique has do a smaller bimedial rectus recession as an initial
resulted in 80% to 85% of patients being aligned sat- procedure for large-angle congenital esotropia and
isfactorily after just one procedure. Other surgeons who decline re-recession of the medial rectus mus-
have equally good results performing bimedial rectus cles. As a rule, a strengthening procedure of a rectus
recessions of up to 7 mm (rarely more) measuring muscle without recession of its antagonist at the same
from the original insertion. However, I continue to procedure is less effective at reducing the angle of
use measurement from the limbus because of the strabismus than a weakening procedure without
wide variations in the medial rectus insertion site and strengthening of the antagonist. Resection is consid-
because of the excellent results obtained with limbal erably less effective when done alone than the same
measurements. resection combined with a recession of the antagonist
When bimedial rectus recession measured from performed at the same procedure. Two situations that
the limbus was introduced, it included routine con- call for bilateral lateral rectus resection are diver-
junctival recession and was called the en bloc or aug- gence insufficiency (paralysis) and residual esotropia
mented recession. Analysis of results indicated that it in a patient who has undergone a maximum bimedial
was unnecessary to routinely recess the conjunctiva. rectus recession. Approximately 20 of esodeviation
We now perform conjunctival recession to the origi- is corrected with a minimal 5 mm bilateral lateral rec-
nal medial rectus insertion site when performing a tus resection and although good data are lacking, I
bimedial rectus recession only in cases where passive estimate that up to 35 to 40 of esotropia could be
abduction is limited preoperatively by a tight con- corrected with a maximum 9 to 10+ mm bilateral lat-
junctiva that occurs in approximately 5% of cases. In eral rectus resection.
110
Workup of the strabismus patient
111
Chapter 4
of exotropic Duane syndrome might be treated with a approximately 20 to 25 of exotropia and produce
single lateral rectus recession to relieve a small angle about the same reduction in the exotropia in the dis-
of exotropia. Usually no more than 15 of deviation tance and at near. A maximum recession-resection
can be corrected with single muscle surgery for procedure for exotropia is 8 to 10 mm lateral rectus
exotropia. An exception would be a larger correction recession and 10+ mm medial rectus resection. This
obtained in case of advancement of a slipped muscle. procedure would be expected to correct up to 50 of
exotropia.
The majority of exotropic patients, in my experi-
ence, have basic exotropia (same exotropia distance
and near) or simulated divergence excess exotropia
Two-muscle surgery for exotropia: (near exotropia equal to or nearly equal to distance
exotropia after several hours occlusion of one eye).
Bilateral lateral rectus recession. A minimum
For this reason, a recession-resection procedure is
bilateral lateral rectus recession of 5 mm will correct
arguably the most logical procedure. However, to
approximately 20 to 25 of exotropia. A maximum
avoid the necessity of resecting the medial rectus
bilateral lateral rectus recession of 8+ mm can correct
muscle, a procedure which may produce a reddened
up to 50 of exotropia. The same angle would be cor-
conjunctiva and sometimes limited abduction, it is
rected if the deviation were either manifest or latent.
common for the surgeon to choose a bilateral lateral
Bilateral lateral rectus recession is a common strabis-
rectus recession in most cases of intermittent
mus surgical procedure.
exotropia.
A recession-resection procedure is an effective
and predictable way to alter the alignment of the eyes
to produce cosmetic as well as functional improve-
ment. Surgically induced incomitance is infrequent
and is not usually significant when it does occur. This
Bimedial rectus resection. Strengthening and
incomitance can be of benefit for patients treated for
weakening procedures of the extraocular muscles
convergence insufficiency because it allows them to
mainly improve ocular alignment while maintaining
find an area of fusion at near and at distance, even
or creating comitance. These procedures do not ordi-
when a small overcorrection occurs, by assuming a
narily influence vergences. Nevertheless, certain
face-turn.
patients demonstrating intractable convergence
insufficiency not helped by near point exercises or
other orthoptic treatment may be helped some with a
bimedial rectus resection. A 5 to 7 mm bimedial rec-
tus resection can be considered reasonable treatment
for a convergence insufficiency measuring between Three-muscle surgery for exotropia:
12 and 25 of exotropia at near and with less or no A maximum bilateral lateral rectus recession of 8
exotropia in the distance and with a remote near point to 10 mm combined with a maximum medial rectus
of convergence. Bimedial resection for convergence resection of 10+ mm in one eye will correct up to 75
insufficiency can result in esotropia in the distance as of exotropia. To correct deviations between 50 and
the price for relieving near symptoms. 75 maximum, three muscle surgery is reduced by
Intermittent exotropia persisting after bilateral 0.5 to 1 mm per muscle.
lateral rectus recession with a significant exodevia-
tion at distance and at near can also be treated with a
bimedial rectus resection. In such a case, a minimum
5 mm bimedial rectus resection can correct approxi-
mately 20 of exotropia. A maximum 10+ mm bilat-
eral medial rectus resection can correct up to 40 of Four-muscle surgery for exotropia:
exotropia.
A maximum 8 to 10 mm bilateral lateral rectus
recession combined with a maximum 10+ mm bime-
dial rectus recession will correct 90 to 100 of
exotropia. If four muscle surgery is indicated for
exotropia, a maximum approach is usually done.
Lateral rectus recession with medial rectus resec-
tion. A minimum recession-resection procedure for
exotropia is 5 mm lateral rectus recession and 5 mm
medial rectus resection. This approach will correct
112
Workup of the strabismus patient
113
Chapter 4
Surgery of the oblique muscles superior oblique that should be tucked depends on
how lax or redundant the tendon is at the time of sur-
gery. This is determined by the superior oblique trac-
tion test (see page 95). This test should be done in
both eyes during every procedure for superior oblique
over or under action. It is done even if other muscles
are treated. During surgery the tendon is brought up
into the tucker or it is tucked free hand until the ten-
don feels taut; at this point the tuck is secured but
with a loop that allows for adjustment. The superior
oblique traction test is then repeated. The tuck is then
Superior oblique weakening: adjusted, if needed, and secured when the traction test
Tenotomy of the superior oblique muscle pro- is equal to or slightly tighter in the treated compared
duces approximately 5 to 15 reduction in the to the normal fellow eye. The size of the tuck may
hypodeviation in the primary position and slightly vary from 6 to 20 mm or more, but it is always dic-
greater reduction in the field of action of the muscle. tated by the preoperative tendon laxity and the
This procedure may be graded somewhat by shifting desired result is an equal or nearly equal superior
the site of the tenotomy closer to the insertion for less oblique traction test. Tuck of a superior oblique ten-
effect or closer to the trochlea for more effect. It may don done in the presence of a normal or tight traction
also be graded with a guard suture which in effect test preoperatively or performing too large a tuck of a
produces a hang-loose tendon lengthening. This was lax tendon will result in an unwanted iatrogenic
called a chicken suture by Phil Knapp. A hang Brown.
loose superior oblique tendon weakening can also be Resection of the superior oblique tendon may be
done from the insertion. If a tenectomy is done, the accomplished at the insertion in a similar amount to
degree of weakening of the superior oblique is prob- the tuck. In our experience, an effective superior
ably not affected by the amount of tendon removed oblique tuck or resection produces some Brown syn-
but instead by the proximity of the nasal margin of the drome or mechanical restriction to elevation in
tenectomy to the trochlea. The fascia in the vicinity adduction, at least in the early postoperative period.
of the superior oblique tendon should be left as undis- Strengthening of the superior oblique is usually per-
turbed as possible when doing a superior oblique formed in superior oblique palsy of the type which
tenotomy in order to achieve more predictable results. has recently been re-classified as congenital versus
In practice, unilateral superior oblique weakening is acquired. Of these two, the congenital palsies have
seldom performed except in the case of Brown syn- been shown to have a high rate of abnormalities of the
drome or superior oblique myokymia. Wright tendon. In most cases these are seen as a redundant
devised a system for weakening the superior oblique tendon. With a loose tendon, a tuck or any other
employing a silicone spacer. Bilateral superior strengthening procedure is safe and effective (see
oblique weakening is more common and is discussed chapter 16).
in the section on surgery for A and V patterns (see Anterior shift of the superior oblique:
chapter 16). In practice, bilateral weakening of the The intorting power of the superior oblique may
superior oblique tends to have greater effect in larger be enhanced by moving all or part of the effective
deviations and less effect in smaller deviations. insertion anteriorly and temporally. This is called the
Harada-Ito procedure. In my experience, superior
oblique tuck or resection improves both the torsional
and vertical deviation. However, when a large tor-
sional deviation of 10 to 15 degrees is present in a
patient who has a small vertical deviation, anterior
shift is a useful surgical tool. Anterior shift has been
done with an adjustable suture technique but this has
not become a popular procedure. Selective disinser-
tion of posterior tendon fibers weakens the depressor
Superior oblique strengthening: effect of the superior oblique, while selective disin-
Strengthening of the superior oblique by a tuck at sertion of the anterior fibers weakens intorsion.
its insertion produces up to 15 reduction in the
hyperdeviation in the primary position and a similar
reduction in the hyperdeviation in the field of action
of this muscle if the tuck is done properly producing
normal symmetric tendon tension. The amount of the
114
Workup of the strabismus patient
115
Chapter 4
resections) or when a recession-resection is per- While patients with mechanical restriction and
formed. The horizontal rectus muscles also may be fusion potential are prime candidates for an
moved vertically without recession or resection in adjustable suture, fusion potential and diplopia are
cases of A or V pattern without oblique dysfunc- not absolute requirements. Cases not suitable for an
tion and when no horizontal deviation is present in adjustable suture are congenital esotropia, intermit-
the primary position. tent exotropia (some may disagree), dissociated verti-
Horizontal shift of the vertical rectus muscles cal deviation, any inferior oblique surgery, and sur-
can be done for treatment of A and V patterns. To gery for convergence insufficiency, etc.
decrease an esodeviation, the vertical recti are shifted An adjustable medial rectus recession may be
temporally and to decrease an exodeviation, the verti- combined with a muscle transfer procedure for treat-
cal recti are shifted nasally. For example, temporal ment of sixth nerve palsy. Some patients may be
shift of the inferior rectus muscles can be done in V adjusted at surgery on the table when local anesthet-
pattern esotropia in patients without a deviation in the ic is used, in the recovery room, or in the clinic on the
primary position and without oblique muscle dys- day following surgery. I have heard one very experi-
function. The best use of this information is when enced surgeon describe adjustment done more than a
recessing the inferior rectus in thyroid disease. Nasal week later! The alignment after adjustment may
shift of these muscles is done to avoid the postopera- remain stable or the alignment may shift over time
tive unwanted A pattern. postoperatively, but this can occur with any type of
strabismus surgery.
The faden operation (posterior Adjustable sutures are useful, but they are only a
fixation suture) small part of my surgical scheme. Some fine strabis-
mus surgeons use an adjustable suture in nearly every
The so-called faden operation should be called
case of rectus muscle surgery. Other surgeons never
posterior fixation suture or retroequatorial myopexy.
use an adjustable suture! Take your choice. When
This procedure has become a regular part of the sur-
used, I prefer the tandem adjustable suture (see page
gical armamentarium for many strabismus surgeons.
259).
It was performed initially for treating the nystagmus
blockage syndrome as suggested by Cppers. Peters Extraocular muscle transfer
described essentially the same procedure earlier, but
it did not catch on. Expanded use of the posterior fix- Extraocular muscle transfer procedures are indi-
ation suture includes any condition in which a sec- cated in complete or near-complete paralysis of a rec-
ondary deviation will promote comitance. This pro- tus muscle. Passive ductions must be free before the
cedure weakens a muscle in its field of action but has transfer is done if optimal results are expected. The
little effect, at least theoretically, on the primary posi- usual procedure in muscle transfer is to shift the
tion deviation or the action of the antagonist of the insertion of the two antagonist rectus muscles to a
operated muscle. In cases of esotropia with nystag- point at or near the insertion of the paretic rectus mus-
mus blockage, the posterior fixation suture is per- cle lying between them. The shift can be carried out
formed alone or is combined with appropriate reces- in some fashion to make up for a paretic medial rec-
sion of the medial rectus muscles. Results of this sur- tus, superior rectus, inferior rectus, or lateral rectus
gery are good, according to some, but when this pro- muscle. Superior oblique tendon transfer is more dif-
cedure is combined with a recession it is difficult to ficult to perform and frequently less effective. It is
know which part of the procedure affects the devia- usually done to reduce the exodeviation in third nerve
tion. palsy. Botox, injection of the antagonist of the paret-
ic muscle can be done in conjunction with a full ten-
Adjustable suture don transfer. Although rare, anterior segment
ischemia can occur when three rectus muscles are
I use an adjustable suture, when indicated, on
detached. Therefore, we like to avoid removing any
any of the rectus muscles and have attempted it with-
extra rectus muscles; Botox can help with this.
out success in a few cases involving the superior
oblique tendon. Indications for use of an adjustable Botox injection
suture are (1) restrictive strabismus in a patient with
fusion potential (the patient with thyroid ophthal- Botox injection remains a viable option for sev-
mopathy may be the prime example of such a eral categories of strabismus. We use it for some
patient); (2) any strabismus in which the outcome of cases of thyroid ophthalmopathy, persistent, residual,
surgery cannot be readily predicted (including or secondary strabismus, some small-angle devia-
patients who had previous unsuccessful surgery); and tions, and in cases where the patient does not wish to
(3) when this technique is expected to produce better have further incisional surgery. We do not use Botox
results according to the surgeons experience. for congenital-infantile esotropia or intermittent
exotropia. Alan Scott has suggested that Botox will
116
Workup of the strabismus patient
be useful for about 15% of strabismus patients. I use 3. The surgeon should aim at a cure with first
it in 3% of cases. A few surgeons use Botox for treat- surgery, provided there are sufficient muscles
ment of congenital esotropia and claim good results. to operate on without causing complications
John Lee of Moorfields in London has more experi- such as anterior segment ischemia.
ence than anyone I know of when it comes to use of 4. Because no procedure is ever 100% success-
Botox for strabismus and he employs it successfully ful, patients (parents) should be given a rea-
in a wide variety of cases. Emilio Campos has report- sonable estimate of the likelihood that a sec-
ed good results after treatment of congenital esotropia ond procedure will be required.
with Botox, but this method of treatment is not used 5. The surgeon should strive toward judicious
widely. boldness and not be excessively fearful of
producing an overcorrection.
Summary of steps 1 & 2 in the 6. If the surgeon is doing the proper amount of
design of strabismus surgery surgery, he or she should expect some over-
When an accurate workup has been completed corrections and should not produce an exces-
and a pertinent history recorded, the surgeon should sive number of undercorrections with regard
possess sufficient knowledge of the patient and the to intended results. For example, if a surgeon
strabismus problem to have certain treatment goals in aims at a 5 undercorrection, a patient who is
mind. In addition, the surgeon should also have real- ortho in the early postoperative period repre-
istic expectations of the results that could be expect- sents a relative overcorrection.
ed from surgery. That is, the surgeon should know 7. More effect is produced per millimeter of
approximately how much change in ocular alignment recession or resection by strabismus surgery
is expected with muscle strengthening, weakening, or in a child or in a patient with a small eye; less
transfer procedures appropriate for the patients stra- effect is produced by strabismus surgery in an
bismus. It is the union of these two factors, 1) patient adult or in a patient with a large eye.
findings and 2) results to be expected from surgery, 8. More effect is gained from strabismus sur-
that enables the surgeon to design each surgical pro- gery on a recent deviation than on a long-
cedure specifically for each patient. This combina- standing deviation.
tion of clinical findings and results expected is made 9. Surgery for a small deviation (25) pro-
more sensitive by the application of certain rules that duces less effect per millimeter of surgery
help the surgeon predict how certain types of patients than that for a large deviation (50).
may respond to strabismus surgery and how selected 10. In patients with cerebral palsy and strabis-
variables could affect the outcome. mus, the more cephalad the neurologic
It should be re-emphasized here that orthoptic, involvement, the longer surgery should be
optical, and to a lesser degree, pharmacologic therapy delayed. Patients with only limb involvement
can be an alternative and in selected cases a better in which the cranial nerves are spared may be
option than surgical therapy for strabismus in select- treated as otherwise normal strabismic chil-
ed patients provided these nonsurgical methods result dren.
in comfortable fusion and the accompanying accept- 11. In partially accommodative esotropia, only
able appearance. Surgery to restore ocular alignment the nonaccommodative part of the deviation
or to enable enhanced binocularity including fusion should be treated surgically.
should be reserved for patients who cannot be helped 12. One 4 mm medial rectus recession corrects
by other nonsurgical means alone. approximately 13 of esotropia.
13. Conjunctival recession is performed when
Step 3: Guidelines for conjunctival scarring causes restricted move-
ment or unsightly appearance.
application of surgical options 14. A minimal recession-resection for either
The third component in the design of the surgical esotropia or exotropia produces approximate-
procedure joins Step 1 (the workup) and Step 2 (sur- ly 25 reduction in the esodeviation or
gical options). The following aphorisms may be exodeviation.
applied to help produce a successful union between 15. A maximum recession-resection procedure
the patient and his or her surgical plan: for either esotropia or exotropia produces
1. If fusion is now or has ever been present, a approximately 50 reduction in the esodevia-
cure with fusion may be expected. A slight tion or exodeviation.
overcorrection may help obtain this result. 16. Three muscle surgery for esotropia or
2. If no fusion potential is present, a slight exotropia may be required for deviations
undercorrection is more likely to produce a between 50 and 75.
stable, small angle residual deviation. 17. Four muscle surgery for either esotropia or
117
Chapter 4
exotropia may be required for deviations rectus or with bilateral lateral rectus reces-
greater than 75 but rarely is performed in sion. Convergence insufficiency is treated
children. with bimedial rectus recession or recession-
18. Esodeviations or exodeviations greater than resection, in one eye.
50 in a patient with very poor vision in one 28. The timing of surgery for intermittent
eye should be treated with a supermaximal exotropia is dictated by how often the devia-
recession-resection of one eye to avoid sur- tion is manifested rather than the deviation
gery on the better eye. measurement.
19. Surgery for esotropia in a hyperkinetic child 29. Once surgery has been decided on for a
produces less effect than the same amount of patient with intermittent exotropia, the
surgery in a placid child. amount of surgery is dictated solely by the
20. Residual esotropia after a bimedial rectus angle of the deviation and is in no way influ-
recession that had been performed several enced by the amount of time deviation is
years before may be treated with re-recession either latent or manifest.
or a marginal myotomy of one previously 30. Bilateral inferior oblique myectomy produces
recessed medial rectus and a resection of one 20 less exotropia or more esotropia in
lateral rectus if the deviation is 25. This upgaze with no significant change of the
procedure should be bilateral if the deviation alignment in the primary position or in
is 50. For deviations between 30 and downgaze.
50, a resection of the lateral rectus alone 31. Unequal bilateral overaction of the inferior
may be performed in the second eye. obliques causing a V pattern should be treat-
21. Residual esotropia occurring weeks to ed with an equal weakening procedure on the
months after a bimedial rectus recession inferior obliques. If only the more overacting
should be treated with a bilateral lateral rectus inferior oblique is weakened, a markedly
resection or a re-recession of the already unequal overaction of the obliques with the
recessed medial rectus muscles. non-operated muscle becoming much more
22. A double 80% marginal myotomy combined overactive may occur postoperatively.
with a resection of the antagonist produces 32. Bilateral superior oblique tenotomy produces
the same weakening effect as a maximum a decrease in exotropia in downgaze between
recession of that muscle. A marginal myoto- 7 and 70. The average change is 30. The
my without resection of the antagonist is a smaller the A, the less the change in
less effective procedure. downgaze; the more the A, the greater the
23. Secondary exotropia after medial rectus change in downgaze.
recession that demonstrates deficient adduc- 33. Vertical shift of the horizontal rectus muscles
tion can be treated with medial rectus resec- for A and V patterns is accomplished as
tion and advancement. follows: the medial rectus muscles are shift-
24. A bilateral lateral rectus recession is per- ed toward the apex of the A or V; e.g. up
formed for exotropia less than 40 that is in A pattern and down in V pattern.
greater at distance with excess abduction and Lateral rectus muscles are moved toward the
equal vision. open end of the pattern; e.g. downward in A
25. Exotropic patients who have lateral incomi- pattern and upward in V pattern. Vertical
tance - that is, who have less exodeviation in shift of the horizontal rectus muscles (one-
lateral versions - tend to be overcorrected half to one muscle width) produces approxi-
more easily than patients whose exodeviation mately 10 to 15 change in the A and V
is the same in the primary position as in later- pattern. The greater the vertical incomitance,
al versions. the more the effect.
26. Exotropic patients who have had extensive 34. Horizontal shift of the vertical recti for treat-
preoperative orthoptics, especially near point ment of A and V is done by shifting the
of convergence exercises, are prone to large muscles temporally to treat esodeviation and
overcorrections after surgery. nasally to treat exodeviation.
27. The choice of muscles to be operated in the 35. Acquired non-traumatic, small angle superior
surgical treatment of intermittent exotropia is oblique palsy in an older patient should be
indicated by the pattern of deviation. evaluated according to the patient's needs.
Divergence excess exotropia is treated with This can be treated with prisms or patching
bilateral lateral rectus recession; basic for 4 to 6 months, and, if necessary, can be
exotropia and simulated divergence excess treated surgically. A medical workup should
exotropia are treated with a recession of the be done.
lateral rectus and a resection of the medial 36. Bilateral superior oblique palsy frequently
118
Workup of the strabismus patient
causes cyclotropia and cyclodiplopia that is 47. DVD with overaction of the inferior obliques
measured at 15 degrees or greater with the and V pattern is effectively treated with
double Maddox rod test. These patients typi- bilateral anterior transposition of the inferior
cally have a chin down, eyes up head posture obliques.
to obtain fusion or wear an eye patch. 48. Congenital superior oblique palsy frequently
37. When a large horizontal deviation and a small demonstrates no torsional response when test-
vertical deviation exist in a patient with no ing with the double Maddox rod test. In addi-
fusion potential only the horizontal deviation tion, spontaneous torsional diplopia is not
is treated surgically. observed. Acquired superior oblique palsy
38. A small vertical deviation in a patient with that is unilateral usually demonstrates torsion
diplopia and fusion potential may be treated with the double Maddox rod test but does not
with surgery and/or prisms. produce spontaneous torsional diplopia. The
39. An unacceptable vertical deviation with or torsion measured in unilateral superior
without fusion potential is treated surgically oblique palsy is less than 15. Both congeni-
by operating on the appropriate vertically act- tal and acquired superior oblique palsy are
ing muscles. The vertical rectus muscles frequently associated with an abnormal head
have more effect on the primary position posture. Bilateral superior oblique palsy
deviation than do the obliques. causes torsional diplopia and torsion greater
40. Brown's superior oblique tendon sheath syn- than 15 degrees with the double Maddox rod
drome is treated surgically only if a cosmeti- test. When greater than 15 degrees of tor-
cally unacceptable vertical strabismus or sional diplopia is found, the diagnosis is bilat-
abnormal head position is present while the eral superior oblique palsy unless ruled other-
patient is fixing in the primary position. wise. In addition, a spontaneous complaint of
41. Lysis of adhesions around an extraocular torsional diplopia is bilateral superior oblique
muscle is usually ineffective unless it is palsy unless ruled out.
accompanied by one or more of the following 49. The superior oblique muscle is the most com-
procedures: conjunctival recession, traction monly occurring anomalous extraocular mus-
suture placement, marginal myotomy, re- cle. If superior oblique palsy that is congeni-
recession, or re-resection. tal is also associated with amblyopia, hori-
42. Replacing tight or scarred conjunctiva to its zontal strabismus, anomalous structure or
preoperative position can nullify the results of even absence of the superior oblique tendon
otherwise potentially successful strabismus should be suspected.
surgery. 50. Superior oblique palsy with facial asymmetry
43. When there is a doubt about whether restrict- is likely to be congenital and to have an
ed motility could be caused by scarred con- anomalous tendon. The larger face is
junctiva, a conjunctival recession should be always on the side of the paretic superior
performed, leaving bare sclera. oblique. The smaller side of the face may
44. Long-term traction sutures should be be characterized by a smaller distance
anchored securely in the sclera or placed in between the lateral canthus and the corner of
the horizontal rectus insertions to avoid the mouth.
unnecessary contact with the cornea. They 51. Superior rectus recession has virtually no
should be tied over a bolster and left in place effect on the palpebral fissure; superior rectus
for several days and watched carefully with resection is likely to narrow the fissure.
the eye fixed in the duction opposite the 52. Inferior rectus recession can significantly
restriction several degrees past the midline. widen the palpebral fissure and inferior rectus
45. The functional improvement of straight eyes resection can narrow the palpebral fissure.
after surgery is compromised when red, 53. Inferior rectus recession is commonly associ-
unsightly scars remain in the conjunctiva. ated with slippage leading to excessive reces-
One should always attempt to retain a normal, sion, especially in thyroid eye disease.
white conjunctiva postoperatively. If this Each surgeon should add his or her own person-
cannot be accomplished otherwise, conjuncti- al guidelines for strabismus surgery to this list and
val recession and/or excision should be per- should delete from this list those aphorisms that do
formed. not apply to his or her experience.
46. Manifest DVD may be treated surgically by
recessing one or both superior rectus muscles
and if the DVD persists resection of one or
both inferior recti should be done.
119
Chapter 4
120
Workup of the strabismus patient
swallowing the medicine and experiencing systemic glasses; and (5) patients with high or relatively high
effect. It is also a good idea to put a drop in one eye hyperopic refractive error will eventually require
in the morning and in the second eye in the afternoon glasses.
to further reduce the chance of an unwanted systemic
effect. If it would be difficult for a patient to return Timing of surgical treatment
for a second visit for an atropine refraction, we add After deciding at what age the infant suspected
phenylephrine HCL 2.5% drops and repeat the of having strabismus is first seen and how amblyopia
Cyclogyl one or two times at 5- and 15- to 20-minute and refractive errors are to be managed, the timing of
intervals, respectively. However, we are satisfied that surgery must be considered. Unless one avoids see-
Cyclogyl is adequate to determine refractive errors in ing children early and/or opposes early surgery on
most infants and children. We do not use 2% theoretical grounds, any delay between completion of
Cyclogyl because of concern about dose-related toxi- nonsurgical treatment (treatment of amblyopia and
city. correction of hyperopia) and surgically straightening
the eyes must be defended. More surgeons are now
Spectacle prescription proceeding to early surgery for congenital esotropia.
Treatment of hyperopia with spectacles in the Surgical straightening of the eyes is done as soon as
esotropic infant can identify refractive esotropia in a amblyopia has been treated and the refractive-accom-
child as young as 6 months. Spectacles should be modative component has been ruled out as the cause
prescribed for all esotropic infants and children with of the esodeviation, in any patient aged six months
greater than +3.00 refractive error. In some cases, and older. Some surgeons, myself included, even
+3.00 glasses or even plus correction of lesser consider four months an appropriate age for surgery
strength is given if it is expected to reduce esotropia. to treat congenital esotropia.
Because this prescription is often not effective and the A factor contributing to my enthusiasm for early
child will need surgery anyway and may not wear surgery is the availability of competent pediatric
glasses afterward, a loaner glasses program can be anesthesia. Safe pediatric anesthesia includes posi-
instituted. Anticholinesterase drops in lieu of glasses tive airway control, use of agents that have a wide
can be used in these children. This medication can be margin of safety, and constant monitoring of heart
used in patients with residual esotropia, particularly if rate, oximetry, blood pressure, respiration, tempera-
a slight reduction in the angle will straighten the eyes ture, and expired CO2. In addition, a continuous
sufficiently to produce improved binocular function. intravenous drip ensures required hydration for the
A more difficult problem is the case of a young preoperatively starved child. Even more important,
child with straight eyes but high hyperopia. For the indwelling intravenous catheter drip allows a
example, we occasionally see a child of 2 or 3 years quick and reliable route for the emergency adminis-
of age with +4.00 hyperopia or greater and straight tration of medication. In the case of older chil-
eyes. No definitive treatment regimen is appropriate dren and adults, the timing of surgery depends for the
in all cases, but some guidelines can be established: most part on the wishes of the patient. When suffi-
(1) if any esodeviation latent or intermittent is detect- cient measurements have been obtained and the devi-
ed, glasses should be given or at least considered; (2) ation has stabilized (as in acute cranial nerve palsy),
if glasses are considered but not given, the parents surgery is offered to the patient and is scheduled at
should be instructed to watch for and report any the most convenient time.
esotropia and an early follow-up appointment should In adults and in cases of acquired strabismus,
be given; (3) if visual acuity is reduced in both eyes surgery is done when nonsurgical methods have
or if in the case of high hyperopia bilateral ametropic accomplished all that they can, the patients health
amblyopia is suspected, glasses should be given; permits, and when the patient wishes.
(4) the higher the hyperopia, the greater the need for
121
5
Diagnostic categories and
classifications of strabismus
This chapter offers a comprehensive classifica- satory head posture. For example, those with con-
tion of strabismus and then provides a detailed genital third nerve palsy, in most cases, never had
description of the more common strabismus types normal fusion, but there is no reason to believe this is
including treatment options. My premise for what why the strabismus is present. On the contrary, motor
could be an unorthodox way to classify strabismus is fusion never developed or was lost because the eyes
that there are only two kinds of strabismus. These are were constantly misaligned because of the cranial
congenital esotropia and its sequelae and all the rest! nerve palsy. Individuals with intermittent exotropia
These two classes of strabismus are divided accord- have excellent fusion interspersed with periods of
ing to the presence or absence of an inborn motor deep suppression. Patients with Brown syndrome or
fusion mechanism (Figure 1). Duane syndrome can have a severe motor dysfunc-
Claud Worth said that congenital esotropia tion that the patient responds to by assuming an
results from a defect in the fusion faculty. Whether anomalous head posture, allowing normal binocular
this so-called fusion faculty refers to sensory fusion vision - fusion. The reason for including in all the
or motor fusion is not agreed upon by experts. rest acquired strabismus such as cranial nerve palsy,
Sensory fusion is the simultaneous perception of refractive esotropia, and mechanical causes such as
slightly different images from the two eyes blending blowout fracture, etc, is obvious.
them into a single object. This object is seen in depth. This more or less arbitrary method of dividing
Motor fusion is the alignment of the visual axes by strabismus has some value in that it helps predict out-
action of the muscles so that an object is seen as one come while guiding treatment. For example, congen-
rather than doubled. This image seen as one by virtue ital esotropia patients are not expected to have normal
of motor fusion may be seen in depth as a result of fusion, regardless of timing and type of treatment,
sensory fusion or may not, at least with clinical tests and are subject to a variety of other strabismus con-
such as the Polaroid vectograph test used in the clini- ditions, most notable of which is dissociated strabis-
cal setting. mus.* In contrast, in the all the rest category many
All the rest of the strabismus entities consist of other types of strabismus, depending on severity and
those who have strabismus, but were born with, or duration, retain the potential for normal or a near nor-
presumably born with the potential for bifoveal mal sensory result after timely and effective treat-
fusion. In this category of strabismus, fusion is lost ment.
from a variety of causes other than a primary inborn Later in Chapter 15 and 16, a wide variety of
deficiency in the central motor fusion mechanism. examples of strabismus will be presented describing
The individuals in the strabismus category, all the clinical characteristics, treatment and results. These
rest, can be said to have either had the potential for will include the more or less naturally occurring types
fusion, but it was lost never to be regained; demon- as well as strabismus from trauma and after prior sur-
strate fusion only part of the time; or appreciate gical treatment.
fusion full time, or nearly so, by assuming a compen-
* Those cases of congenital esotropia who are said to have normal fusion do not, in my opinion, have by definition congenital esotropia.
123
Chapter 5
124
Diagnostic categories & classification of strabismus
Congenital esotropia
The most common form of strabismus is an about the time of onset. The term essential has been
esodeviation with onset during infancy occurring in added to make it clear that we do not know the cause
an otherwise neurologically normal infant. It is of this esodeviation. A paucity of information about
labeled congenital, infantile, or essential infantile ocular motility in the normal newborn as well as in
esotropia. These patients can have other physical the newborn and infant with early onset esodeviation
signs including face turn and nystagmus. Although left strabismologists with several descriptive terms,
the clinical characteristics of this strabismus entity but little understanding of mechanisms.
have been thoroughly described, many questions In the past ten years, new information has
remain about: timing of onset, etiology, terminology, accrued from study of ocular motility in the normal
and treatment outcome. This is understandable newborn. In addition, a national collaborative study
because, for the most part, the earliest stages of this of the clinical behavior of esotropic infants beginning
strabismus have been recorded mostly by parents or at just a few weeks of life has determined that the
pediatricians. These esotropic infants had not been diagnosis of congenital esotropia can be made with
subjected to careful study in large numbers in the confidence at 4 months. This study also suggests to
past by the ophthalmologist or the basic scientist me that surgery can be done as early as four months
studying infant psychophysics. of age to treat a constant esodeviation. Supported by
Now this is changing. The issues surrounding this type of information, surgery is now being done
the causes and early findings in congenital esotropia on infants as young as four months and in some cases,
are being discussed in a new light. The competing younger. Data generated from this experience has
ideas that congenital esotropia is a primary defect of also provided information about the best treatment
the fusion faculty (Worth) or that this condition results that can be obtained. Now based on my own
develops as the otherwise normal binocular system clinical experience and on interpretation of available
is overcome by esotropital factors causing a sec- clinical and laboratory data, I will present my view of
ondary esotropia (Chavasse) are being subjected to the broad clinical picture or what I will call congeni-
scrutiny in the laboratory and the clinic. tal esotropia.
Whether the so-called fusion faculty of Worth is
related to sensory fusion or motor fusion has never Terminology
been clearly stated, but careful reading of Worth indi- The term congenital esotropia was popular-
cates, at least to me, that he was referring to motor ized by Costenbader. However, the word congenital
fusion. This suggests that adequate treatment of con- has been challenged because this condition is not con-
genital esotropia in the form of surgical realignment firmed at birth, except, in some cases, by parents. In
of the eye can improve alignment leading to excellent other words, the esotropia is not conatal, at least as
appearance and peripheral fusion or subnormal binoc- confirmed by expert observation. To counter this
ular vision but treatment cannot achieve normal objection it could be pointed out that other conditions
motor or what depends on normal motor fusion, nor- termed congenital are not necessarily conatal. For
mal sensory function. example, so-called congenital subluxation of the hip
The theory of Chavasse suggesting that a time- is not manifest in most cases until several months
ly reversal of esotropital factors could result in nor- after birth. However, it is arguable to label something
mal fusion although not in my opinion correct, was as congenital if the hidden precursor of a later mani-
the impetus for earlier surgery for congenital festing condition is said to be present at birth. This
esotropia. This was championed by Frank may be the case with congenital esotropia. As an
Costenbader and later Marshall Parks. Their efforts alternative, the term infantile esotropia has been
supported by improved surgical anesthesia, finer used because it more accurately describes the time of
suture, sharper-finer needles, better instruments, and onset of the esodeviation, that is, during infancy, beg-
effective magnification, and carried on by a cadre of ging the question When did the underlying cause
well-trained young strabismologists has resulted in originate? Discouraging use of infantile esotropia,
many ophthalmologists treating congenital esotropia Lang and Parks defend the term congenital
successfully with surgery at 6 months and even esotropia. Parks stressed that it is an established
younger. Some have even claimed near perfect motor term describing an entity whose clinical characteris-
and sensory results in a few cases. These cases tics and response to treatment are well known to all
notwithstanding, the best results from treatment of strabismologists.
congenital esotropia seems to be better but not per- Supporting use of the term infantile esotropia,
fect and then only with close postoperative follow up von Noorden said, I prefer...infantile esotropia to
and appropriate intervention. describe a constant deviation with a documented
The terms congenital and infantile have been onset during the first six months of life and add the
vigorously debated mostly because of uncertainty prefix essential to emphasize the unknown origin
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Chapter 5
and to distinguish it from other forms of esotropia In addition, it must be noted that the infant has either
that occur at birth or during infancy. While I could alternation, often with cross fixation or fixation pref-
defend the term infantile esotropia, I am convinced erence for one eye, implying amblyopia. Other com-
that affected infants have an inborn defect in motor mon clinical findings often present but not essential
fusion. to the diagnosis are: manifest nystagmus, oblique
Is this controversy regarding terminology muscle dysfunction, dissociated strabismus either
important? vertical deviation (DVD) or a horizontal deviation,
primarily an exodeviation of one eye (DHD), variable
The crux of the argument regarding the validity
angle, latent nystagmus, manifest latent nystagmus,
of the terms congenital versus infantile may
and torticollis.
depend on when the initial defect leading to the stra-
All patients with congenital esotropia have
bismus occurs, rather than the timing of the manifest
compromised sensory functioning because the object
esotropia itself. The question is: Does the infant
of regard is seen by the fovea of one eye and nasal
who ultimately is diagnosed as congenital or essen-
retina of the other eye. Stated another way, anatomi-
tial infantile or simply infantile esotropia start life
cally corresponding parts of the visual system will
with the potential for normal binocular vision and
always be seeing something different. This is the
lose it because of acquired motor induced factors, or
basis for development of anomalous retinal corre-
does the infant begin life with an inborn lack of nor-
spondence, which in my opinion is always harmo-
mal binocularity because of a central defect that even-
nious (equal to the objective angle of strabismus) if
tually causes an esodeviation by a failure to provide a
tested with a minimally dissociating stimulus. Non-
template in the central nervous system on which the
harmonious anomalous retinal correspondence, in my
normal binocular motor fusion develops? This may
opinion, is a testing artifact, but this finding may have
be nothing more than a restatement of the arguments
some use as a measure of the depth of adaptation.
surrounding the Worth (fusion faculty) versus
Late sequelae of congenital esotropia with and
Chavasse (motor reflexogenic) theories for the ori-
without treatment include some or all of the follow-
gins of infantile esotropia.
ing: secondary exotropia (large angle, late occur-
Whether the esodeviation is called congenital
ring), DVD, DHD, amblyopia, overaction of the
or infantile may be considered unimportant provided
inferior obliques with V pattern, overaction of the
the etiology remains unknown. On the other hand,
superior obliques with A pattern, X pattern with the
the design of more effective treatment may depend on
overaction of all obliques, and recurrent esodeviation
better understanding of etiology. von Noorden stated,
with or without the influence of a refractive or accom-
If etiology is implied, terminology is important,
modative component. Individuals with congenital
and I tend to agree. Hereafter in this section, using
esotropia have asymmetric optokinetic nystagmus
von Noordens argument but coming to a different
characterized by a normal nystagmus beat for nasally
conclusion, I will use the term congenital esotropia.
directed targets and an abnormal response to tempo-
Characteristics rally directed targets both before and after treatment.
Ciancia has also demonstrated a preponderance of
According to results from the Pediatric Eye response from crossing (nasal retinal) optic nerve
Disease Investigator Group (PEDIG), congenital fibers when comparing hemispheric visually evoked
(infantile) esotropia can be confirmed by a reliable potential (VEP) response in congenital esotropia.
observer by 4 months of age. The minimum required I have examined several patients with most of
findings for diagnosis are as follows:* the typical characteristics of congenital esotropia, but
1. Esotropia--usually 40 to 50 diopters, but with no esotropia! One patient was seen initially
with a range of 10 to 90 prism diopters. under a year of age with DVD but normally aligned
2. Normal neurologic status (except for stra- eyes. She was followed until age 7 years at which
bismus) time accurate sensory testing could be completed.
3. Refractive error expected for age (usually This patient at this time had DVD, asymmetric OKN
low to moderate hyperopia), correction of refractive esotropia and only gross stereo acuity.
which does not eliminate esotropia. Another, a teenage boy, the sibling of a congenital
4. Asymmetric optokinetic nystagmus charac- esotropia patient, had DVD and OKN asymmetry, but
terized by robust temporal to nasal response his eyes were aligned and he demonstrated normal
and erratic nasal to temporal response. stereopsis! Patients with these characteristics have
* An infant with 40 prism diopters or more of constant esotropia at 4 months has a 100% chance of having esotropia at 7 months. If at
4 months the esotropia is less than 40 prism diopters or is intermittent, the likelihood of there being a constant esotropia at 7 months is
70%.
It is unlikely that true overaction of the oblique muscles exists. Instead this could be over expression because of a weak antagonist
or deficient adduction allowing increased expression of the secondary abducting action of the oblique muscles.
126
Diagnostic categories & classification of strabismus
127
Chapter 5
128
Diagnostic categories & classification of strabismus
During the course of follow up, two patients to temporal. This response seems to be a common
required hyperopic correction to maintain alignment* denominator of congenital esotropia. That is, all con-
and two patients required a short period of occlusion genital esotropia patients demonstrate this and con-
for amblyopia. At the end of the follow up period 19 versely if a patient demonstrates asymmetric OKN
of 20 eyes had visual acuity of 20/40 or better. All response, he/she has congenital esotropia.
patients were aligned to within 10 prism diopters of Looking at the results of surgical treatment for
orthotropia at distance, near, or both. Nine of 10 congenital esotropia reported by seven investigators,
patients had dissociated vertical deviation and 4 had the following becomes clear: (Table 6)
latent nystagmus. Four patients had measurable 1. Stereo acuity is attainable, but it is reduced.
stereo acuity, two at 3,000 seconds (stereo fly), one at 2. Most patients require at least one additional
400 seconds, and one at 140 seconds (Table 5). surgery.
All of the patients demonstrated optokinetic 3. Dissociated vertical deviation occurs in most.
asymmetry which is characterized by smooth pursuit It has been suggested that early surgery in the
of stripes moving from temporal to nasal and jerky patient with congenital esotropia might lead to a high-
eye movement response to stripes moving from nasal er incidence of dissociated vertical deviation. To test
* Both had less than +3.00 refraction before surgery. The patient who was +3.00 before surgery was plano at the last visit.
129
Chapter 5
this, Neely, et. al. in a retrospective study demon- The dynamic nature of congenital esotropia is
strated that DVD developed approximately 18 demonstrated by the logs of four patients in this study.
months after surgical alignment in congenital A record of each visit along with preoperative pic-
esotropia patients aligned both early and at a later tures and a picture at the end of follow up show the
time. This study concluded that the timing of surgery satisfactory cosmetic results obtained in these
for congenital esotropia had no bearing on the occur- patients after a total of 97 visits and 17 interventions
rence of DVD. Rather, DVD occurs about one and a (including surgery, patching, and prescription of
half years after surgery for esotropia, regardless of the glasses). (Tables 7-10)
surgery being done early or later.
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Diagnostic categories & classification of strabismus
Table 7 Case #5
From Helveston EM, et al. Results of early alignment of congenital esotropia. Ophthalmology, 1999, 106(9):1716-1726.
Used with permission.
Table 8 Case #1
From Helveston EM, et al. Results of early alignment of congenital esotropia. Ophthalmology, 1999, 106(9):1716-1726.
Used with permission.
131
Chapter 5
Table 9 Case #7
From Helveston EM, et al. Results of early alignment of congenital esotropia. Ophthalmology, 1999, 106(9):1716-1726.
Used with permission.
132
Diagnostic categories & classification of strabismus
133
Chapter 5
134
Diagnostic categories & classification of strabismus
135
Chapter 5
A B
Figure 6
A Before 2 months of age, the motor and sensory systems B Completed arch between 2 and 4 months.
are immature.
136
Diagnostic categories & classification of strabismus
Infantile esotropia with nystagmus as a promi- esotropia. In spite of having at least 20/20 vision in
nent feature may be an example of a combined motor each eye and no more than 2 prism diopters of phoria,
and brain stem--derived esotropia. Nystagmus block- these parents had a 16% incidence of reduced stereo
age type esotropia with manifest latent or manifest acuity of 80 sec arc or less and could be classified as
nystagmus may represent a secondary defect having subnormal binocular vision. This was in con-
(esotropia) occurring after convergence to damp nys- trast to a control group of parents who had a 2% inci-
tagmus. Those cases of congenital esotropia without dence of subnormal binocular vision. Adding to these
manifest nystagmus may be the only ones with a true statistics is the well-known fact that children with
congenital, occipital cortex--based, motor-sensory congenital esotropia are more likely to have a parent
fusion defect. or first-order relative with esotropia compared to chil-
When incomitant strabismus is present in a dren without congenital esotropia. Following this
patient with normal motor fusion potential, it can be study, we found no difference in the response to treat-
dealt with by assuming a head posture which results ment in those children with or without a parent with
in alignment of the eye. In these cases, it is the head subnormal binocular vision. This suggests to us that
that moves while the eyes remain fixed with fusion on defective stereo acuity is a subtle inborn defect which
the object of regard. Examples of this strategy could be a form fruste of congenital esotropia, but the
employed in Brown, superior oblique palsy, and true relationship between a minimal defect in stereo-
Duane are shown in Figure 10. acuity and normal alignment remains unexplained.
I believe that the presence of the keystone for On the other hand, asymmetric OKN is a reaction to
motor and ultimately sensory fusion is genetically ocular misalignment even as mild as minimal DVD
determined. Likewise, the absence of this keystone is and is therefore classified as a result, not a cause, of
genetic. The presence or absence of this characteris- congenital esotropia. The stereo acuity defect is
tic cannot be uncovered by clinical means at our dis- inborn and cortically based. The OKN defect is sec-
posal until approximately two to four months of age ondary and brain stem based.
when the system is programmed to complete the Another way to look at the picture of congeni-
scheme for the development of binocular alignment. tal esotropia in a graphic way and still retain the ele-
ments of sensory (afferent), motor (efferent), and sen-
Possible hereditary factors sory-motor fusion (link or keystone) components of
In an effort to learn more about possible hered- infantile esotropia is to picture a closed loop system
itary factors in congenital esotropia, we studied oth- (Figure 11).
erwise normal parents of children with congenital
137
Chapter 5
B B1
C C1
D D1
Figure 10
A By changing the foundation, the arch can maintain its C Duane syndrome, right eye
integrity provided the keystone of motor fusion is C1 This girl with Class II Duane turns her head toward the
intact. This is done when patients assume an appropri- normal side to achieve fusion.
ate head posture in: D Right superior oblique palsy
B Mild Brown syndrome, left eye D1 A chin down, head tilt left is the head posture assumed
B1 Eyes are aligned when head moves to position where to gain fusion by this child with congenital right superior
strabismus is maximum in Brown. oblique palsy.
138
Diagnostic categories & classification of strabismus
B C
Figure 11
A The nine components of the complete closed loop of C Congenital esotropia with a pathologic open loop.
binocular vision. D Mature normal loop.
B The immature open loop demonstrating unstable align-
ment.
139
Chapter 5
The occurrence of associated finding with con- Ciancia syndrome is esotropia with limited
genital esotropia has been described according their abduction and manifest latent nystagmus of the
salient features by von Noorden (Table 11). He also abducting eye. These patients fixate with the adduct-
offers a differential diagnosis (Table 12). Other ed eye and turn their face to the side of the fixing eye.
descriptions of infantile esotropia have been offered This is called cross fixation. The primary congenital
by Lang, Adelstein, Cppers, and Ciancia. Harcourt defect is in the cortical motor fusion center, seen as
simply described congenital esotropia with and with- esotropia with manifest latent nystagmus secondary
out nystagmus. I prefer to recognize Ciancia syn- to brain stem response.
drome as congenital esotropia that has manifest latent Nystagmus blockage syndrome is manifest
nystagmus as the most prominent feature. Nystagmus congenital nystagmus damped by convergence. Both
blockage syndrome is manifest congenital nystagmus eyes are crossed. One eye is used for fixation and the
with a null in convergence. While several important face is turned toward the fixating eye. The primary
characteristics are associated with different types of defect is presumed to be in the brain stem.
esotropia in infancy, important clinical features of Latent and manifest latent nystagmus, dissoci-
congenital esotropia are strabismus (always),OKN ated vertical deviation, and amblyopia can occur as
asymmetry (always), nystagmus, and abnormal head later findings in any esotropia of infancy. In my
posture (sometimes). These features can be seen with experience, congenital esotropia without nystagmus
some or all of the findings listed as characteristics of is more likely to be aligned with one procedure con-
congenital esotropia in Table 11. sisting of an appropriate size bimedial rectus reces-
Early refractive esotropia, not included in this sion. Sprunger, et. al. have shown that in the presence
scheme, is usually easily distinguishable because of a
later onset, better fusion potential, and response to
correction of hyperopia. Other characteristics of con-
genital esotropia including amblyopia, DVD, and
oblique muscle dysfunction are secondary. The prin- Essential infantile esotropia*
cipal characteristics of esotropia in infancy can be Sixth nerve palsy
combined in a Venn diagram, which makes the rela- Nystagmus dampened by convergence
tionships of the early onset esodeviations easier to Esotropia with central nervous system anomalies
understand (Figure 12) (Table 13). (Downs syndrome, albinism, cerebral
Primary congenital esotropia has a moderate palsy, mental retardation, and the like)
to large angle esotropia. The patient may alternate or Refractive accommodative esotropia
prefer fixation with one eye (amblyopia). Sensory esotropia
Nystagmus, manifest or latent, and anomalous head Duane syndrome, type 1
posture are usually not present at the outset but may * congenital esotropia
occur later. The primary defect is presumed to be a
Mbius syndrome (EMH)
congenital defect in the central, cortical motor fusion
center.
Table 12 Differential diagnosis of infantile esotropia
according to von Noorden
140
Diagnostic categories & classification of strabismus
Manifest jerky nystagmus that decreases or disappears with esotropia, accommodative convergence
when sustaining fixation on an object distance or near.
Ciancia syndrome
141
Chapter 5
of nystagmus, it is more difficult to achieve alignment the fact that moderate amounts of uncorrected hyper-
in congenital esotropia. Both Ciancia syndrome and opia are usually accompanied by accommodation to
nystagmus blockage syndrome are more likely to achieve a clear image, and this would automatically
require additional surgery to achieve horizontal align- invoke accommodative convergence with the poten-
ment. Duane syndrome I is a truly congenital tial for elimination of or reduction of the exodevia-
esotropia but has a normal sensorimotor status and tion. Others have found a normal distribution of
with the proper head posture, in most cases, fusion is refractive errors in patients with intermittent
obtained. Surgery in cases of Duane syndrome is exotropia. For whatever reason, those patients I have
ordinarily done to improve head posture and primary treated for intermittent exotropia tend to have very lit-
position alignment. Even with successful surgery, tle, if any, hyperopia.
some strabismus remains.
Age of onset
Intermittent exotropia Clinically significant intermittent exotropia can
be seen in infants 6 months of age or even younger. A
Intermittent exotropia is a common, though more common age of onset is during the toddler peri-
enigmatic, form of strabismus that presents only a od, between 1 1/2 and 3 years of age. Parents may
modest diagnostic challenge but which requires con- observe that their child does not look at me. It is
siderable therapeutic acumen and tenacity. Patients usually difficult for parents to accurately describe this
with this type of strabismus, more than any other, type of strabismus. They often say that the eyes of the
demonstrate a duality of behavior in that they seem to child simply dont look right. At other times, par-
be completely normal during orthotropic interludes ents have said that they have seen their childs eyes
and totally turned off during periods of manifest turn in. After careful questioning it usually becomes
exodeviation. At the outset it should be stated clear that the parents are describing the recovery
exophoria; that is, a latent and usually small-angle movement from the exodeviation to the straight posi-
exodeviation, is not amenable to surgical treatment. tion--therefore, the turning in. The pattern of exode-
This latent deviation, which by definition is kept in viation over time is usually characterized by a steady
check under binocular conditions, can cause symp- progression with increasing periods of tropia during
toms of asthenopia best treated with orthoptic exer- the preschool years. However, in some cases the
cises, but not prism wear which will be eaten up. deviation remains stable and a few even improve.
These exercises can enhance motor fusion amplitudes Paradoxically, it has been shown by Archer that giv-
to control the deviation in both phoric and some ing full correction in some hyperopic patients can
intermittently tropic patients. In such cases, the lead to control of intermittent exotropia.
greater the motor fusion response for convergence,
the more effectively the deviation will be held in Pattern of deviation in the young
check with reduction or elimination of symptoms. Two characteristics of the intermittent
Orthoptic exercises can consist of near point of con- exotropia deviation in the preschool years are
vergence push up maneuvers, used for convergence (1) increasing likelihood of tropia when the child is
insufficiency that has a greater exodeviation at near, fatigued, day-dreaming, or inattentive and (2) closing
convergence enhancement with fusible targets, work- one eye in bright sunlight. The latter is an especially
ing against base-out prism, and use of over-minus interesting phenomenon. von Noorden and Wiggins
lenses that invoke accommodative convergence. have shown that fusional amplitudes decrease in the
In contrast to the exophoric patient, those majority of subjects, both strabismic and normal,
patients with intermittent exotropia who are tropic when tested with increasing illumination. It is also a
demonstrating a manifest deviation part of the time commonly observed phenomenon that most normal
and who require surgical correction occur in several people when in bright sunlight outdoors will close the
forms. They are classified on the basis of when the eye nearer to the sun, while unconsciously using the
eyes are deviated and in what field or distance of gaze nose in part to shade the fixating eye. If the sunny-
the deviation is greater. However, a strong undercur- side eye were exotropic, it would be all the more
rent of similarity connects all forms of intermittent exposed to the sun and therefore demanding closure
and latent exodeviations. for comfort. It is important to elicit this history of
closing one eye in bright sunlight when confirming a
Refractive error in intermittent diagnosis of intermittent exotropia in a young child.
exotropia When this question is asked of a parent and they
Patients with intermittent exotropia in our expe- respond in the affirmative, it lends a measure of cre-
rience have a low refractive error, either plus or dence to you, the examiner, and tends to confirm that
minus, with or without moderate astigmatism. This the exodeviation is present during these periods.
may be a valid observation, but it may also be due to
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Diagnostic categories & classification of strabismus
143
Chapter 5
downgaze. The rules of treatment are consistent. If exodeviated or even straight during cover testing, at
oblique dysfunction is a cause of the A or V pattern, the expense of vision, which is fogged, though unde-
the obliques may be weakened. However, the superi- tected, by accommodative convergence.
or oblique should be weakened only if other surgical To obtain an accurate distance measurement
considerations such as downshift of the lateral or some prefer to measure while a patient fixates on a far
upshift of the medial rectus muscles would be con- distant object at infinity. To do this, the patient may
sidered ineffective, if no exodeviation is present in be asked to look out of a window at a distant chimney,
primary position and, of course, if definite overaction clock, tree, etc., while prism and cover testing is car-
of the superior oblique is demonstrated. Bilateral ried out to detect the maximum distance exodevia-
superior oblique weakening should be avoided in tion.
most A pattern patients who demonstrate fusion. In A short-term use of a patch can differentiate
most cases of intermittent exotropia with A or V, I simulated from true divergence excess. Use of alter-
prefer when possible to perform appropriate vertical nate patching for days or weeks in patients with inter-
shift of the horizontal recti, sometimes without reces- mittent exotropia has been said to result in a reduction
sion if no deviation is present in the primary position. in the exodeviation, but I have not employed this
X patterns will usually resolve with correction of the technique often.
primary position deviation after surgery done only on
the horizontal recti. I have not found it necessary to Parental observation
weaken all of the obliques, as has been suggested. During the initial examination of a child with
A, V, or X pattern deviations are more likely to intermittent exotropia, an important part of the
be seen in constant than in intermittent tropias. process is to actually demonstrate the deviation to the
parents. They should be shown the misalignment of
Combined horizontal and vertical the eyes just after dissociation and while the eyes are
deviation with intermittent exotropia exotropic. This is described to them as the devia-
If when the exodeviation is neutralized with tion. Next, the family observes the recovery move-
base-in prism during the prism and cover test the ver- ment. It has been important in our practice to send
tical deviation is eliminated, the vertical deviation families home from an initial examination of a child
(which may be called a dissociation vertical) may with intermittent exotropia with instructions to keep a
be ignored at the time of surgical correction. On the report card of their childs ocular alignment. At
other hand, if the vertical deviation persists after the subsequent examinations parents should report
horizontal deviation has been neutralized with prism, approximately how often the childs eyes are deviat-
it deserves to be treated with appropriate vertical rec- ed, under what conditions the childs eyes are deviat-
tus or inferior oblique surgery. Persisting vertical ed, how readily the childs eyes can be straightened or
deviation is more likely to occur with constant exode- recovery takes place, etc. Without this type of
viation. When combined vertical and horizontal sur- demonstration parents may bring such a child for
gery is done, this surgery usually consists of superior repeated examinations without ever really under-
rectus recession on the appropriate side. standing the true nature of the deviation!
144
Diagnostic categories & classification of strabismus
Symptomatic convergence insufficiency may be Most surgeons prefer to establish a course of progres-
treated with base-in prism. While of theoretical sion of the intermittent exotropia and then preform
value, I have not used weak or incomplete cyclople- surgery at the mid-preschool years, between 2 1/2 and
gia to stimulate accommodative convergence. 4 years of age or even later. However, exceptions to
this rule do occur; I have operated on a few patients
Surgical treatment of intermittent with intermittent exotropia who were under the age of
exotropia 1 year of age. In older children and adults with basic
intermittent exotropia, and adults with convergence
The surgical treatment of intermittent exotropia
insufficiency, surgery is done when the patient wish-
presents some philosophical problems. These
es, either because of problems with appearance or
patients have a lot going for them before anything is
from asthenopia, or both.
done. Vision is usually equal and normal in each eye,
sensory fusion with stereopsis and motor fusion Choice of muscles and amount of
amplitudes are present, and versions and ductions are
intact. In other words, the good intermittent
surgery
exotropia patient is very, very good. On the other Basic intermittent exotropia and pseudodiver-
hand, at times when the eyes are exodeviated, central gence basic intermittent exotropia are treated the
field binocular cooperation seems to be turned off. same way. The main choices surgically are between
One eye drifts out, suppression of the central field is bilateral lateral rectus recession and lateral rectus
profound, and that part of the visual experience is recession combined with medial rectus resection.
essentially ignored. The motility and sensory condi- While individual surgeons may have strong prefer-
tion in intermittent exotropia is certainly dynamic, ences for one technique. No strong evidence exists to
varying between having one turned off exodeviated support one technique over the other.
eye and having perfectly normal binocular coopera- For most cases, I prefer bilateral lateral rectus
tion. At the same time, a static surgical procedure is recession over recession-resection. The reason for
used for treatment. By static I mean that muscles are this is that the recession procedure is slightly more
recessed or shortened (resected) to alter an alignment physiologic than the resection procedure. In addition,
that is orthotropic much of the time. There is no evi- medial rectus resection can produce a lump under the
dence that this surgery has any specific effect on ver- conjunctiva. For larger deviations, it is necessary to
gences or fusional ability. With this apparent illogi- add to bilateral lateral rectus recession a medial rec-
cal application, the fit is not always perfect. Some tus resection or even bimedial rectus resection.
flexibility in treatment plan and diligence in the fol- A useful table for surgical numbers follows:*
low-up, especially with regard to nonsurgical inter- Bilateral lateral rectus recession
vention as well as surgical treatment, is necessary. 5.0 mm OU/20-25 prism diopters
6.0 mm OU/25-30 prism diopters
Timing of surgery 7.0 mm OU/30-40 prism diopters
Strabismologists disagree on the best time for 8.0 mm OU/40-50 prism diopters
surgery. Some prefer early surgery for intermittent Recession lateral rectus--resection medial
exotropia, saying that it is important to avoid pro- rectus
longed periods of suppression that can lead to deteri- 5.0 mm--*5.0 mm/20-25 prism diopters
oration of the normal fusion substrate. On the con- 6.0 mm--*6.0 mm/25-30 prism diopters
trary, others believe it is safe to follow these children 7.0 mm--*8.0 mm/30-40 prism diopters
with observation, stressing that patients are as good as 8.0 mm--*10.0 mm/40-50 prism diopters
they are at their best; the glass is half full, not half Three-muscle surgery
empty. Two arguments for delay are (1) overcorrec- 8.0 mm--*6.0 mm--8.0 mm/50-60 prism
tion is easier to deal with in an older, more coopera- diopters
tive patient and (2) some intermittent exotropia 8.0 mm--*8.0 mm--8.0 mm/60-75 prism
patients remain the same and a few even improve. diopters
The great fear in surgical treatment of intermittent Four-muscle surgery
exotropia is overcorrecting a very young patient and 8.0 mm--*8.0 mm--*8.0mm/70-85prism
converting this patient into a small-angle esotropia diopters
with loss of stereopsis and possibly producing ambly- 8.0 mm--*10.0 mm--*10.0 mm--8.0
opia. The majority of strabismologists do not consid- mm/85-100 prism diopters
er intermittent exotropia in the young patient between In most cases of intermittent exotropia requir-
ages approximately 1 and 3 years to be an emergency. ing surgery, the deviation is 50 prism diopters or less.
* Any time you read surgical numbers in this book, be aware that these are only my best approximation - a place to start. Each sur-
geons numbers must be his or her own! The second asterisk refers to medial rectus resection.
145
Chapter 5
Therefore, two-muscle surgery--either bilateral later- sary, these may be replaced with permanent prisms.
al rectus recession or recession of one lateral rectus Prolonged prism treatment of overcorrected intermit-
and resection of the same medial rectus muscle of the tent exotropia can produce excellent results. One
same eye--is sufficient. patient who was initially undercorrected with bilater-
al lateral rectus recession had bimedial rectus resec-
Divergence excess intermittent tion as a second procedure that produced 14 prism
exotropia diopters of esotropia postoperatively. She wore
steadily decreasing base-out prisms for three years
This condition is best treated with bilateral lateral rec-
before reverting to orthophoria with normal stereop-
tus recession. A table for surgery follows:
sis. Her father, a university professor of philosophy
5.0 mm OU/20-25 prism diopters
anxiously followed this long drawn out process, mak-
6.0 mm OU/25-30 prism diopters
ing it unforgettable. It is rare that a patient requires
7.0 mm OU/30-40 prism diopters
reoperation for overcorrection in cases where surgery
8.0 mm OU/40-50 prism diopters
has been done properly and ductions are full.
This surgery is done without regard for the near
However, incomitant esotropia occurring after sur-
deviation. In most cases, correction of the distance
gery for intermittent exotropia, especially after reces-
deviation has no adverse effect on the near deviation.
sion and resection has been done, may require reces-
However, in two very intense, highly motivated,
sion of a tight medial rectus muscle or advancement
teenage female patients the distance deviation could
of a weak lateral rectus muscle.
be repaired only at the expense of the near deviation.
Undercorrection of intermittent exotropia calls for
One patient requested to have her eyes over-corrected
reoperation based on the alignment with knowledge
to an esotropia when she was in college so that she
of the muscles previously operated. In general, if the
could read. She wore base-out prism for driving.
laterals have been recessed less than maximum, they
After she finished school and began to work as a
may be re-recessed. If the laterals have been recessed
nurse she did the opposite--that is, wanted her eyes
maximally, the medials may be resected, etc.
placed straight in the distance while she wore base-in
prism for reading at near. Convergence insufficiency
Convergence insufficiency intermittent exotropia is
Results of surgery for intermittent an entirely different situation. I have had some suc-
exotropia cess in treating this with bimedial rectus resection,
The treatment results for intermittent exotropia are but in other cases results have been disappointing,
not as easy to assess as in other forms of strabismus with virtually no change in alignment after surgery
treatment. In general, success is defined as convert- even in cases where an early overcorrection occurred.
ing an intermittent tropia to a phoria. Simply reduc- The amount of bimedial rectus resection should be
ing the angle of a persisting intermittent tropia is not small and symmetrical, ranging from 4 to 6 mm.
much help since the same sensory pattern, suppres- Some prefer a small recess-resect procedure to treat
sion when tropic, persists. In addition, the size can convergence insufficiency. Although this could cause
increase postoperatively. the patient to assume a slight face turn at near to
A desirable, but also sometimes worrisome, early maintain comfortable single binocular vision while
postoperative result is to convert an intermittent reading, the reward is that the patient can find an area
exotropia into a constant small angle esotropia. of fusion because the deviation is incomitant.
These patients experience diplopia postoperatively,
which may be treated with patching for a short time Does intermittent exotropia progress
or base-out prism if a longer period of treatment is to constant esotropia?
needed. These overcorrections eventually revert to
stable surgically corrected status in most cases. Any Intermittent exotropia can proceed to a constant devi-
patient (or parent) must be alerted to the fact that ation in some cases. This may be a reason to perform
diplopia from postoperative esotropia can be a good surgery in a timely manner. Perhaps in no other kind
sign. These patients are also given full plus glasses if strabismus is the surgeon more obligated to make
they have any hyperopia. Patients may also be given informed decisions ahead of time and to follow the
phospholine iodide to reduce accommodative conver- patient diligently postoperatively instituting appropri-
gence. ate and timely re-treatment. It cannot be overstressed
This treatment of the postoperative esotropia is con- that these patients are completely normal during peri-
tinued for several weeks or months. If the esotropia ods of alignment. The surgeon is obligated to reme-
with diplopia persists, it is treated with fully correct- dy the condition occurring when the patient is abnor-
ing Fresnel base-out prism on their present glasses or mal but to leave undisturbed the alignment present
on plano loaner glasses which are provided. If neces- during periods of normalcy. Herein lies the challenge
of the surgical treatment for intermittent exotropia.
146
Diagnostic categories & classification of strabismus
147
Chapter 5
syndrome are in marked contrast to patients with increased normally in upgaze during elevation in
Duane syndrome who tend to become increasingly adduction. During a period of 10 years, I treated 59
troubled by their strabismus in adulthood, far out of patients with tuck or resection of the superior
proportion to what seems a minimal deviation while oblique. Nine of these 59 or 17% had a postoperative
patients with congenital Brown syndrome are not par- Brown syndrome so severe that a second surgery was
ticularly bothered as adults. needed to take down the tuck. Since that series of
When treating congenital Brown syndrome sur- cases, I have learned about the wide variation in the
gically we do a cuffed limbal incision, hook the superior oblique tendon, especially in congenital
superior rectus, and inspect the superior oblique ten- superior oblique palsy. With this knowledge, superi-
don carefully, beginning at the insertion and continu- or oblique tuck and resection are done on a more
ing to the trochlear cuff. In most cases, in order to selective basis, being reserved for congenital superior
have better exposure, it helps to disinsert the superior oblique palsy with a loose or anomalously inserted
rectus and replace it later. Forced ductions are repeat- tendon confirmed by the superior oblique traction test
ed often during the process of identifying the tendon and reconfirmed at surgery.
to determine the cause of limited elevation. The best Traumatic Brown syndrome is difficult to treat.
treatment for congenital Brown varies according to The scarring associated with trauma in the area of the
the surgeons experience and may be one of the least superior oblique tendon and trochlea is difficult if not
agreed upon aspects of strabismus surgery. This is impossible to totally eradicate to produce free move-
not too surprising because freeing a mechanical ment because of the mechanical restriction which
restriction while simultaneously maintaining ade- usually affects both up- and downgaze. Traumatic
quate rotations in all directions may be the strabismus Brown syndrome coexisting with superior oblique
surgeons greatest challenge. Most surgeons believe underaction has been called canine tooth syndrome
that the least amount of surgery that will free ductions by Knapp. It is due to (1) local trauma restricting
is the best. For example, one surgical option is disin- both upgaze and downgaze, (2) local trauma restrict-
sertion of the posterior seven-eights of the superior ing upgaze and fourth nerve palsy restricting
oblique insertion at least as a first try. If this frees downgaze, or (3) iatrogenic Brown syndrome restrict-
passive ductions at surgery, no more surgery is done. ing upgaze and residual fourth nerve palsy restricting
This works very well in a few cases, probably downgaze (Figure 13).
because the posterior-medial fibers of the tendon have Before undertaking surgery for Brown syn-
mainly a vertical effect, but the procedure can fail drome, the surgeon must first confirm the diagnosis
with return of the Brown in spite of demonstrating by demonstrating restricted forced ductions at eleva-
free forced ductions at the conclusion of surgery. tion in adduction then:
Other surgical options, all involving the superior 1. Make sure there is a good reason for under-
oblique complex, include freeing the fascia surround- taking surgery, such as diplopia, hypertropia,
ing the tendon, recession of the tendon, tenotomy or a bothersome head posture
near the insertion, tenotomy between the superior rec- 2. Prepare the patient to have lowered expecta-
tus and trochlea, and use of a silicone expander. All tions
techniques have their advocates. The fact that there 3. Be ready for a possible second surgery,
are so many choices leads to the undeniable conclu- including treatment for iatrogenic superior
sion that no specific alternative is the best for all oblique palsy, especially if a superior oblique
cases. tenectomy is done.
Regardless of how the superior oblique is 4. Do not weaken the inferior oblique at the first
weakened, the risk of postoperative superior oblique procedure.
palsy exists. Crawford found this frequently. Parks 5. Remember that the little people grow up,
suggests simultaneous inferior oblique weakening to meaning that restricted upgaze could become
treat the presumed superior oblique palsy caused by less of a factor.
superior oblique weakening to treat the Brown syn- 6. Apply any other good points that experience
drome. However, Sprunger et al., found only a third has taught.
of patients having superior oblique tenectomy for
Brown syndrome had superior oblique palsy requir-
ing inferior oblique weakening. They suggest doing
inferior oblique weakening at a second procedure and
only if needed.
Iatrogenic Brown syndrome results from exces-
sive shortening the superior oblique tendon when
treating superior oblique palsy with a resection or a
tuck. It occurs because the distance between the
trochlea and the superior oblique insertion cannot be
148
Diagnostic categories & classification of strabismus
or inferred
149
Chapter 5
side and the eyes toward the opposite side. In most able head posture, severe up- and downshoot of the
cases, these patients are able to recognize normal or affected eye in adduction, and severe enophthalmos.
near-normal stereopsis and rarely have amblyopia. The most common type of Duane syndrome--the type
Enophthalmos with fissure narrowing usually occurs with moderate esotropia in the primary position along
in the involved eye only during attempts to extreme with face turn toward the involved eye--is best treat-
adduction. Abduction in the involved eye is typically ed with a small recession of the medial rectus of the
just beyond the midline. Upshoot and downshoot are involved eye. Other types of Duane syndrome are
not a prominent feature. treated with the aim of aligning the eyes in the pri-
Other patients with Duane syndrome are either mary position. It is a good rule to avoid resection of
orthotropic in the primary position or have an the lateral rectus muscle in Duane syndrome. This
exotropia in the primary position. The primary posi- can lead to worsening of enophthalmos and up- and
tion alignment seems to depend on the tightness or downshoot. Some have advocated modified full ten-
tone of the lateral rectus muscle. The tighter the later- don transfer of the superior and inferior rectus to the
al rectus muscle the less esotropic the patient (the lateral rectus to increase the field of binocular vision.
straighter or more exotropic the involved eye), and I have not done this procedure, but it has been done
the greater the enophthalmos on attempts at adduction safely and there could be indications for doing this.
in the involved eye. These patients with a tight later- When up- and downshoot are the main problems, it
al rectus are also more likely to have up and/or down- may be appropriate to recess both the medial and lat-
shoot of the involved eye in adduction. This holds eral recti of the involved eye and also the medial or
true except in patients who have a very large-angle lateral rectus of the fellow eye. Other techniques for
exotropia with Duane syndrome. These patients can treating the up- and downshoot include posterior fix-
demonstrate simultaneous abduction when looking ation suture of the lateral rectus muscle to keep it
toward the sound eye. This is presumably due to the from slipping upward and downward and also Y split
mechanical advantage assumed by the lateral rectus of the insertion of the lateral rectus muscle (Table 14).
during co-contraction with the eye already widely When treating Duane syndrome, it is essential
exodeviated. This could be called class IV. to know and also to inform the patient that this con-
The upshoot or downshoot in Duane is not due dition cannot be eliminated; however, the signs and
to over- or undercorrection of the oblique muscles but symptoms can be improved by appropriate surgery.
is instead caused by a taut wire effect with the eye
either slipping above or below the midline under the Practical classification of Duane
influence of the extreme tension produced by medial syndrome
rectus contraction against a co-contracting lateral rec-
tus. The extreme type of exotropic Duane syndrome Esotropic Duane - Huber I
with simultaneous abduction was originally called 1. Esotropia with head straight
perversion of the extraocular muscles. It is more 2. Face turn to involved side
accurately described as simultaneous abduction. This 3. Limited abduction
condition is rare, but I have seen a half dozen cases. 4. Near normal adduction
Regardless of the variations in clinical appear- 5. Mild enophthalmos and fissure narrowing
ance, all Duane syndrome patients appear to have a on adduction (but may be severe)
common etiology, just a varied expression. The con- 6. Sensory examination usually normal
dition seems to be slightly more prevalent in girls and
in the left eye. It is usually unilateral but may be Exotropic Duane - Huber II
bilateral. In bilateral Duane the rules for head posi- 1. Face turn toward normal side
tion do not follow. Duane syndrome has been 2. Limitation of adduction and no or min-
described as a genetic condition occurring in siblings imal limitation of abduction
and in consecutive generations. Duane syndrome is 3. Marked upshoot and downshoot on
also associated with craniofacial-mandibular cleft attempted adduction
anomalies including Goldenhar and Wildervanck syn- 4. Enophthalmos and fissure narrowing on
dromes. Children with Duane syndrome rarely com- attempted adduction, usually with up-
plain. They are usually brought in for examination shoot and downshoot
because of the head turn or strabismus, but often par- 5. More likely to suppress
ents are really unsure about the specific problem.
They simply suspect that there is something wrong. Straight Duane - Huber III
On the other hand, adults with Duane syndrome often 1. Limited abduction and adduction
complain bitterly of asthenopia, intermittent diplopia, 2. Marked narrowing of fissure on attempt
and a general feeling of being ill at ease. ed adduction with enophthalmos
Indications for surgery for Duane syndrome 3. Upshoot and downshoot on attempted
include strabismus in the primary position, unaccept- adduction
150
Diagnostic categories & classification of strabismus
Diagnosis Surgery
Esotropic Duane with severe enophthalmos I Recess medial and lateral recti of involved eye, medial
rectus recession or posterior fixation suture on contralat-
eral eye if larger esotropia
Straight-eye Duane with up- and downshoot III Recess medial and lateral recti of involved eye if enoph-
thalmos severe; consider Y split of lateral rectus or poste-
rior fixation suture of lateral rectus (both with or without
recession)
Exotropic Duane II Recess lateral rectus of involved eye; can add Y split;
may require larger lateral rectus recession of uninvolved
eye.
Simultaneous abduction IV Recess both lateral recti; emphasis on the involved side;
may need to resect medial rectus of involved eye
* Extraocular muscle transfer shifting the vertical recti adjacent to the lateral rectus insertion of the involved eye has been done and may
be a viable option for mild esotropic Duane.
Table 14 Duane treatment guidelines
151
Chapter 5
The fourth nerve nucleus is in the rostral part of Brown syndrome after superior oblique strengthen-
the brain stem in the tectum. The nerve fibers emerge ing.
from the nucleus dorsally and decussate. The fibers Double Maddox rod torsion. Seeing a tilted
then pass through the tentorium as delicate fibrils. line on testing with double Maddox rod is very sup-
They course into the orbit through the superior orbital portive of the diagnosis of superior oblique palsy.
fissure where they have as their sole purpose the This usually means that the superior oblique palsy is
innervation of the superior oblique muscle. These acquired.
delicate fibrils are vulnerable to violent to-and-fro Overaction of the contralateral superior
motion of the brain, such as occurs with a sudden oblique. Underaction of the ipsilateral inferior rectus
deceleration in an automobile accident or similar (so-called fixation duress because it is working
head trauma. against a contracted antagonist) and overaction of the
contralateral superior oblique, its yoke, occurs in
Patient presentation longstanding superior oblique palsy with contracture
History. The patient or parents of the patient of the ipsilateral superior rectus.
frequently either describe an acute event (such as a Double Maddox rod torsion greater than 15
motor vehicle accident) that is likely to be the cause degrees. This is a strong indication of bilateral supe-
of a traumatic fourth nerve palsy or report a history of rior oblique palsy.
diplopia, asthenopia, or anomalous head posture, Bielschowsky head tilt test. This test is consid-
often present for many years or for life. Occasionally, ered positive for superior oblique palsy when the ver-
old pictures demonstrating a head tilt and chin tical deviation increases with the head tilted toward
depression are useful in supporting the diagnosis of the higher eye. If the Bielschowsky head tilt test
congenital superior oblique palsy. reverses, then a bilateral superior oblique palsy is sus-
Head posture. The usual head posture in supe- pected. If the Bielschowsky head tilt test does not
rior oblique palsy is the head tilted to the opposite reverse but is reduced to no or very little hypertropia
side with the chin depressed. Actually, the head in the same direction on tilt to the side opposite the
moves where the eye cannot be moved by the paretic paretic superior oblique, a masked bilateral superior
superior oblique. This is the rule when fusion poten- oblique palsy may be suspected.
tial is present in a person with incomitant strabismus. Fundus torsion. Torsion may be noted during
With the head in this posture, the eyes look upward examination with the indirect ophthalmoscope. If the
and to the opposite side, completely out of (opposite) macula is rotated downward or clockwise in the left
the field of action of the paretic muscle. In a very eye and counterclockwise in the right eye, so that the
small percentage of patients, the head posture may be macula is below a line drawn parallel to the orbit
in the opposite direction, presumably to maximize the floor and temporal from the lower disc margin, tor-
separation of diplopia and make it easier to suppress. sion can be inferred. This is confirmed if the macula
Torticollis. Neck contracture can occur in very is also shown to be roughly equidistant between the
young children with superior oblique palsy. temporal arcades while in its lower position.
However, neck contracture from superior oblique Inhibitional palsy of the contralateral antago-
palsy does not occur before the child sits up and/or nist. When the eye with the paretic superior oblique
walks. The head tilt from superior oblique palsy does is used for fixation the yoke inferior rectus in the con-
not occur with the child supine or prone but only tralateral eye receives extra innervation. Its antago-
when the child is vertically oriented, sitting, standing nist, the superior rectus and also the levator palpebri
up or walking. on that side are inhibited resulting in hypotropia and
Motility. Versions are an extremely important more importantly, pseudoptosis. When the normal
part of the diagnosis of superior oblique palsy. The eye takes up fixation, the ptosis disappears.
most tell-tale finding is inferior oblique overaction, Other indicators.
and to a lesser extent superior oblique underaction, 1. Diagnostic position prism and cover testing is
which occurs to varying degrees. Sometimes the more important for quantification of the deviation
superior oblique underaction is slight or undetectable. than it is for diagnosis.
Diplopia. Vertical diplopia is a common com- 2. Facial asymmetry is seen commonly in cases of
plaint in adult patients but rare in children. congenital superior oblique palsy. The face is
Asthenopia is also common in adults. This may take always fuller on the side of the paretic muscle.
the form of neck ache while reading. The reason for this is the abnormal head posture
Chin depression. In the presence of a V pattern assumed to maintain single binocular vision.
the chin is often down. This occurs with bilateral 3. Horizontal strabismus can occur in addition to the
superior oblique palsy. superior oblique palsy.
Cyclodiplopia. Spontaneous complaint of 4. Amblyopia in the presence of congenital superior
cyclodiplopia is a common sign of acquired bilateral oblique palsy may indicate an abnormal or even
superior oblique palsy. It also occurs in iatrogenic absent superior oblique.
152
Diagnostic categories & classification of strabismus
When the preceding considerations have been Congenital superior oblique palsy
dealt with and when full measurements have been
completed, especially prism and cover testing in the There is usually no history of trauma.
diagnostic positions, double Maddox rod testing, and The condition is long-standing and character-
the head tilt test, it is possible to diagnose, classify, ized by a large head tilt and supported by fam-
and establish a treatment plan for a patient with supe- ily pictures showing a head tilt.
rior oblique palsy. In cases of absence of the superior oblique ten-
don, amblyopia and horizontal strabismus are
Acquired superior oblique palsy common.
Facial asymmetry is common in all types of
Patients with acquired unilateral palsy will usu- congenital superior oblique palsy. The face is
ally have the following characteristics: fuller on the involved side.
A discrete history of onset There is frequently no torsion measured with
Complaint of intermittent vertical diplopia the double Maddox rod.
Head tilt and chin depression with a comment, There are fewer complaints of diplopia in con-
I see better if I assume this head position genital compared to acquired superior oblique
Measurable torsion with the double Maddox palsy.
rod, less than 15 degrees In the operating room, patients with congenital
A vertical deviation usually less than 20 prism superior oblique palsy a frequently found to have a
diopters (deviation may be greater at near and lax superior oblique traction test (see page 97). At
in longstanding cases). exploration of a superior oblique tendon that was
Bilateral acquired superior oblique palsy differs found to be loose or lax with the traction test, an
in that a V pattern is the rule; single vision is more anomalous superior oblique tendon will be noted to
likely to occur in upgaze with chin down; be either too long, inserted in the wrong place, or
Bielschowsky test is bilaterally positive; that is, right absent.
hyper with right tilt and left hyper with left tilt or the
hyper may disappear or nearly so on head tilt to one Superior oblique treatment
side; and the Maddox rod frequently shows a classification
cyclotropia of greater than 15 degrees.
Unilateral superior oblique palsy from a Treatment is based on prism cover measure-
microvascular accident is usually much smaller ment findings, torsion, and the results of superior
amplitude than unilateral superior oblique palsy from oblique traction testing indicating the state of the ten-
trauma. These patients usually have a vertical devia- don. Hatched areas shown in the diagrams on the fol-
tion in the neighborhood of 5 to 10 prism diopters and lowing pages represent the field of greater deviation
are older, being more often in the seventh or eighth and assume left superior oblique palsy. The pattern of
decade, and they complain of diplopia. They may not deviation is the examiners view.
demonstrate a head tilt. These patients deserve a med- The scheme described here is that proposed by
ical/neurological work up for hypertension, diabetes, Philip Knapp in 1971. It remains, with a few modifi-
etc. cations, valid today (Figure 14).
In the operating room, patients with acquired
superior oblique palsy, either unilateral or bilateral,
will usually be found to have a normal superior
oblique tendon on the traction test. The tendon is
very easily felt, and the traction test is usually bilater-
ally symmetrical in unilateral disease.
153
Chapter 5
Class I Class II
Knapp I--overaction of antagonist inferior Knapp II--underaction of the paretic superior
oblique with deviation about 20 prism diopters or less oblique with the deviation greater in the field of
in the field of action of the antagonist; this is a com- action of the paretic superior oblique seen mostly in
mon pattern for both acquired and congenital superi- smaller angle, acquired microvascular superior
or oblique palsy oblique palsy. This is best treated with prism and
Surgery. Weaken antagonist inferior oblique. time. A larger angle deviation with this pattern can
This is the safest surgical procedure for any superi- occur in congenital absence of the superior oblique
or oblique palsy. tendon. If a pattern like this emerges in a congenital
superior oblique palsy with facial asymmetry and
pronounced superior oblique underaction, superior
oblique traction testing followed by exploration of the
superior oblique will lead to the appropriate surgical
plan which could include superior oblique tuck, infe-
rior oblique weakening, or yoke inferior rectus weak-
ening depending on the angle and the state of the
superior oblique.
154
Diagnostic categories & classification of strabismus
Class VI
Bilateral superior oblique palsy
This condition is characterized by:
1. History of trauma
2. Spontaneous torsional diplopia
Class IV 3. Usually >15 degrees torsion with double
This common pattern of hyperdeviation which Maddox rod testing
is class III demonstrates a spread of hyperdeviation 4. V pattern
across the bottom occurring because of tightness of 5. Reversing Bielschowsky (or nearly revers-
the ipsilateral superior rectus. ing Bielschowsky) test
Surgery. If the deviation is 20 prism diopters Surgery. There is little agreement among
or less, weakening of the antagonist inferior oblique experts when it comes to surgical treatment of bilat-
and ipsilateral superior rectus is effective. If the devi- eral superior oblique palsy. Bilateral weakening of
ation is greater, the superior oblique tendon can be the yoke inferior obliques is favored by some to treat
tucked if it is loose or the yoke inferior rectus can be the V and the torsion. Others do a bilateral reces-
recessed if the superior oblique tendon is normal. sion of the inferior recti. The strength of either pro-
cedure is that the weakening is done on a normal mus-
cle, one is a yoke and the other an antagonist.
Bilateral weakening of the antagonist inferior
obliques likewise treats the V and the torsion, but
depends on getting more out of a paretic muscle.
Antero-lateral shift of the superior oblique (Harada-
Ito) treats the torsion. For the V, downshift of the
medial recti can be done. The superior oblique ten-
don should not be tucked.
155
Chapter 5
156
Diagnostic categories & classification of strabismus
8. Be aware of the possibility of masked bilater- established congenital disease, or less clear presumed
al superior oblique palsy. If the Bielschowsky microvascular disease in an elderly individual. In the
head tilt reverses, or nearly does, be suspi- last instance, an evaluation by an internist for hyper-
cious. Either treat this as a unilateral superi- tension and/or diabetes is needed. Extensive imaging
or oblique palsy and expect to do a second with CT or MRI or lumbar puncture and EEG studies
procedure or do two muscles on the more rarely accomplish anything useful for the usual supe-
involved side and one muscle on the masked rior oblique palsy patient. In my opinion, extensive
side according to the scheme presented. testing of the patient with fourth nerve palsy should
Work-up of a patient with acquired superior be done only if indications other than the fourth nerve
oblique palsy should in most cases be kept to a mini- palsy itself are noted. By that I mean other significant
mum. The etiology is usually clear-cut trauma, well- neurologic signs or symptoms.
Figure 15
Description of the superior oblique tendon in anatomic congenital superior oblique palsy.
157
Chapter 5
TABLE I: PATIENT DEMOGRAPHICS AND PREOPERATIVE DATA FOR 190 CASES OF SUPERIOR OBLIQUE PALSY
Sex
Male: 105
Female: 85
Age
Range: 6mo-79yr
Mean: 28.8+/-22.2 yr
Mean for congenital group: 24.1+/-21.1 yr
Mean for acquired group: 40.9+/-20.5 yr
Refraction
Mean: -0.49+/-3.04 diopters
Visual Acuity
Mean: 20/25
Median: 20/20
Congenital/acquired
Congenital: 137
Acquired: 53
Origin: Trauma 29
Iatrogenic 12
Vascular 7
Tumor 5
Knapp Classifications
Class I: 28
Class II: 13
Class III: 65
Class IV: 53
Glass V: 5
Class VI: 19
Class VII: 1
Class VIII: 6*
Laterality
Right: 92
Left: 79
Bilateral: 19
Facial asymmetry
Present: 56 51 congenital 5 acquired
Absent: 69 40 congenital 29 acquired
Unknown: 65 46 congenital 19 acquired
Abnormal head posture:
Right tilt: 55
Left tilt: 70
Others (eg, head turn, chin down): 10
No abnormal head posture: 39
Unknown: 16
Forced duction tests
Tests performed: 161
Tendon laxity: 95 83 congenital
12 acquired
No tendon laxity: 66 37 congenital
29 acquired
Table 15
Patient demographics and preoperative data for 190 cases of superior oblique palsy.
From Helveston EM, et al. Surgical treatment of superior oblique palsy. Transactions of the American Ophthalmological
Society, Vol. XCIV, 1996, pp. 315-334. Used with permission.
158
Diagnostic categories & classification of strabismus
Table 16
From Helveston EM, et al. Surgical treatment of superior oblique palsy. Transactions of the American Ophthalmological
Society, Vol. XCIV, 1996, pp. 315-334. Used with permission.
159
Section 3
Techniques of exposure
Conjunctival incision
Overview
The conjunctival incision for strabismus surgery of these steps must be accomplished before attempt-
has two main requirements: 1) it should provide ade- ing to engage the extraocular muscle on a hook. Once
quate exposure to the muscle(s) to be operated on, sclera has been identified, the tip of the muscle hook
and 2) it should avoid excessive scarring and leave slightly indents sclera at the muscle border and is then
the conjunctiva in the palpebral opening white and passed gently behind the muscle insertion, or in the
smooth after healing has taken place. A technique for case of the inferior oblique, behind the muscle's belly.
incising the conjunctiva satisfying the first require- Any impedance of the passage of the hook behind the
ment was devised by Swan who described doing muscle insertion suggests that the hook is in the
extraocular muscle surgery beneath Tenon's capsule. wrong plane. Clean engagement of the extraocular
With this technique, conjunctiva and anterior Tenon's muscle on the muscle hook is necessary for the start
capsule are incised isolating the muscle in the plane of successful strabismus surgery
of posterior Tenon's capsule. This tissue layer makes
up the intermuscular membrane and the muscle's cap- Start of the surgical procedure
sule. Posterior Tenon's capsule is incised separately to Surgery on the extraocular muscles starts with
expose sclera. This provides access to the muscle in the placement of a lid speculum between the lids for
a physiologic "compartment" lying between anterior exposure to the front of the eye. A light, adjustable
Tenon's capsule and sclera. Swans contribution was solid bladed speculum is ideal. A standard adult size
significant because it introduced a logical approach to is used for adults and a pediatric size for children (see
the tissue planes around the rectus muscles. The Chapter 2). With the speculum in place, it is now
drawback is that the Swan incision is made over the possible to see the insertion of the rectus muscles
muscle's insertion and thereby in the palpebral open- through the conjunctiva in most cases. These appear
ing where it could heal as a raised, reddened ridge. beneath the conjunctival surface as a subtle elevation
The techniques currently used for conjunctival slightly darker than the surround. Rotation of the eye
incision like that of Swan adhere to the principle of at this point enhances the view of these muscles and
operating beneath anterior Tenon's capsule, but they also in some cases the anterior ciliary vessels further
differ in location of the initial incision through con- delineating the rectus muscles. Seeing these muscle
junctiva and in the degree of exposure of the muscle. insertions aids in orientation for the placement of the
Techniques achieving suitable exposure of the incision, especially when the eye has undergone
extraocular muscles include the following: torsion as it sometimes does with general anesthesia.
1. Transconjunctival incision in the cul-de-sac Next, it is important to perform forced or passive
(Parks) ductions in abduction, adduction, elevation and
2. Limbal incision in the palpebral opening depression testing for restrictions. The superior
3. Retropalpebral transconjunctival incision oblique traction test is done if there is any question
4. Superior cuffed limbal incision about laxity of the superior oblique tendon (see page
All of these techniques have in common an incision 97).
through conjunctiva, anterior Tenon's capsule, and
posterior Tenon's capsule exposing bare sclera. Each
163
Chapter 6
A B
Figure 1
A Four-0 black silk sutures are placed in episclera at the B Four-0 black silk sutures are placed in episclera at the 3
12 o'clock and 6 o'clock limbus for traction to rotate the o'clock and 9 o'clock position to rotate the eye upward or
eye medially or laterally for exposure with the limbal downward for exposure with the limbal incision.
incision.
A B
Figure 2
A locking forceps is placed at the limbus of the right eye grasping conjunctiva and episclera to rotate the eye:
A down and in to expose the superior temporal quadrant; C up and out to expose the inferior nasal quadrant prior
B up and in to expose the inferior temporal quadrant; to the cul-de-sac incision to expose the medial rectus.
164
Mechanics of surgery
A B
Figure 3
A The locations of the limbal incision for exposure of each C Locations of the conjunctival incision for exposure of the
of the rectus muscles. oblique muscles: 1) inferior oblique, 2) superior oblique
B The locations of the cul-de-sac incision. The most useful (trochlea) nasal to the superior rectus, 3) superior oblique
one is in the inferior nasal quadrant for exposure of the tendon at the insertion.
medial rectus.
properly. While these disadvantages may sound over the muscle. Disadvantages of the cul-de-sac
daunting, they can be managed easily with sufficient incision are the following: 1) it is more difficult to
care on the part of the surgeon. Those who routinely perform; 2) exposure is less than with other incisions
use the limbal incision can manage the bleeding when especially the limbal incision. The view with the cul-
it occurs and tend to become skilled at conjunctival de-sac incision has been described as peek a boo; 3)
closure. Above all, these surgeons appreciate the there is no opportunity for recession of the conjuncti-
excellent exposure leading to what some believe is va; 4) fragile conjunctiva such as is present in older*
more accurate surgery. The limbal incision has the people can tear as the incision is stretched over the
advantage of making postoperative handling of an muscle's insertion; 5) postoperative handling of an
adjustable suture much easier. This incision is also adjustable suture can be more difficult.
important in that it allows for removal of scarred and After listing all of these disadvantages, it
unsightly conjunctiva in some cases of reoperation, should be stated clearly that the cul-de-sac incision is
and most important the limbal incision allows for ideally suited for use with recession of the medial rec-
recession of tight, restrictive conjunctiva, with or tus muscle in young children. The thick Tenon's cap-
without scarring, in cases of strabismus influenced by sule and healthy conjunctiva in the young stretch
these mechanical factors. readily over the muscle insertion providing good
Cul-de-sac incision exposure for surgery. At the conclusion of the proce-
dure, the young firm tissue slides behind the lid and
The cul-de-sac incision devised by Parks has
remains hidden completely if the procedure has been
the advantage of being hidden behind the lid. (Figure
done properly. Observing such a patient immediately
3B) In addition, it usually requires no suture for clo-
after surgery it may be difficult to detect that surgery
sure at least inferiorly where it is used most. The
has been done. This appearance changes slightly a
patient may be more comfortable in the immediate
few hours later when some swelling usually becomes
postoperative period if no excessive swelling occurs
* In this case, older can refer to patients as young as late teens or early twenties.
165
Chapter 6
evident. Although the cul-de-sac incision can be used Unique characteristics of each
to expose the lateral rectus and is also used superior- extraocular muscle in terms of initial
ly, the usefulness in these locations is far less than for exposure after the conjunctival
exposure of the medial rectus, especially in the incision
young.
Conjunctival incision for exposing oblique muscles Each of the rectus muscles has its own special
The conjunctival incision to expose the inferi- characteristics or personality. The surgeon should
or oblique is made in the middle of the inferior tem- have knowledge of this in advance for the best chance
poral quadrant about 8 mm from the limbus. It is of success at any surgical undertaking.
essential that this initial incision be posterior to the Medial rectus
line of insertion of posterior Tenon's capsule that
Of the rectus muscles, the medial rectus inserts
describes the spiral of Tillaux. An incision so placed
closest to the limbus. In the esotropic patient this dis-
goes through conjunctiva, anterior Tenon's capsule,
tance can vary from 3.0 to 6.0 mm with the stated nor-
and posterior Tenon's capsule (intermuscular mem-
mal being 5.5 mm. This variation in insertion site,
brane) exposing bare sclera after which the inferior
along with the fact that there seems to be no relation-
oblique can be seen as it passes in posterior Tenon's
ship between the distance of the insertion from the
capsule.
limbus and the angle of esotropia in esotropic
A similar conjunctival incision can be made
patients, is a reason for measuring medial rectus
just medial to or just lateral to the insertion of the
recession from the limbus rather than the original
superior rectus, but also posterior to the insertion of
insertion. The medial rectus has no other muscle or
posterior Tenon's capsule to expose the superior
orbital fascial structure associated with it other than
oblique medial to the superior rectus, near the
the intermuscular membrane to stop its retraction
trochlea, or at the insertion of the superior oblique
back into the orbit when it is detached. For this rea-
(Figure 3C).
son, the medial rectus is the most likely of any of the
Conjunctival incision and reoperation rectus muscles to fall back into the orbit if a suture
Reoperation can be done after either a limbal or breaks causing what is referred to as a slipped or
a cul-de-sac incision. The ease of re-operation in lost muscle.
either case depends more on the care exercised by the When looking at the orbital surface of the
original surgeon and the particular nature of healing medial rectus muscle, the origin of anterior Tenon's
in the individual than on the type of incision. capsule can be seen as it joins the medial rectus cap-
sule. Just outside of anterior Tenon's capsule, toward
the medial orbital wall, is the medial rectus pulley. If
a hole is made in the undersurface of anterior Tenon's
capsule at this point fat will prolapse. It is also possi-
ble with more dissection to disengage the medial rec-
The ideal conjunctival incision should have the tus from its pulley. Disruption of fat is to be avoided!
following characteristics: This can cause unwanted adhesions and worse.
Muscle immersed in fat after surgical intervention
1. Minimal scar in the palpebral opening can result in the muscle dissolving! Surgeons differ in
after surgery their approach to the intermuscular membrane when
2. Adequate exposure operating on the medial rectus. Minimal dissection of
3. Ease of performance the tissue seems to be the best choice in my opinion.
4. Absence of excessive adhesions In summary, surgery on the medial rectus should be
between Tenon's capsule, muscle sheath, carried out anterior or distal to the origins of anterior
and sclera Tenon's capsule with avoidance of any fat prolapse.
5. Ease of reoperation The surgery should avoid disruption of the pulleys
6. Able to allow relaxation of restrictive unless this is a stated aim as might be so with a mus-
scar tissue cle transfer procedure. Minimal dissection of inter-
7. Allows excision of excessive scar tissue muscular membrane should be carried out unless
8. Postoperative comfort there is a specific reason to do otherwise.
9. Allows for postoperative adjustment Superior rectus
when called for. This muscle is located farthest from the limbus
of the rectus muscles and has the broadest insertion.
It is, in my experience, the most difficult of the rectus
muscles to engage on the muscle hook, especially
when introducing the hook from the temporal side. It
is common to unintentionally split this muscle inser-
166
Mechanics of surgery
tion. When attempting to hook the muscle from lower lid retraction. Others place a suture in this tis-
either side, especially temporally, be certain to identi- sue tying it forward on the globe to keep Lockwood's
fy bare sclera posterior to the insertion of posterior and the lower lid retractors forward and thereby
Tenon's capsule. Once engaged on the muscle hook, reducing the retraction effect on the lower lid. The
it becomes clear that fat is not likely to be encoun- unique relationship of the inferior oblique and inferi-
tered, even with intermuscular membrane dissection or rectus may contribute to destabilization of the
more than 10 mm posterior to the superior rectus inferior rectus-scleral union causing this muscle to
insertion. The superior oblique tendon is fused to the slip after it has been detached and reattached at sur-
undersurface of the superior rectus by a common gery. This occurs far more frequently with the inferi-
capsule. If this connection is not severed it should or rectus than other rectus muscles. A less important
reduce the effect of a large recession of the superior but nonetheless present occurrence is the elevation of
rectus done for treatment of dissociated vertical devi- the lower lid with narrowing of the fissure with infe-
ation. This is disputed by some who claim the supe- rior rectus resection.
rior rectus remains recessed the very large (intended) Inferior oblique
amount in spite of not being freed from the underly-
The inferior oblique is engaged in the inferior
ing superior oblique. Recession of the superior rectus
temporal quadrant through an incision made just ante-
can cause retraction of the upper lid and widening of
rior to the mid portion of the distal half of the muscle.
the palpebral fissure and, conversely, resection of the
The belly of the inferior oblique is embedded in
superior rectus can cause forward movement of the
Tenon's capsule and must be shelled out. This is not
upper lid and narrowing of the palpebral fissure.
difficult, but care must be exercised to avoid splitting
Lateral rectus the muscle. At the mid portion of the exposed mus-
The lateral rectus muscle is easy to hook and is cle a large vortex vein is seen exiting sclera and enter-
usually found, as stated in anatomy books, 6.9 mm ing the orbit passing through Tenons. This is a near-
from the limbus. On the lower border of the lateral ly 100% occurrence. Exposure of the inferior oblique
rectus and about 12 mm from the muscles insertion is helped by triangulating the opening by placing a
the anterior corner of the inferior oblique insertion is muscle hook behind both the lateral and inferior rec-
found. This is an important relationship. Surgery on tus muscles and then using a retractor to pull back the
the lateral rectus, either recession or resection, can posterior edge of the conjunctival incision with
result in inadvertent inclusion of the anterior fibers of Tenons included. Inferior oblique anatomy is quite
the inferior oblique which attach along the lower bor- reliable although there have been reports of cases of
der of the lateral rectus. This usually goes unrecog- bifid insertion.
nized at the time of surgery. The result is an unex- Superior oblique
pected postoperative hypodeviation, hyperdeviation
The superior oblique muscle is not seen during
or exodeviation or a combination of these. Simply
strabismus surgery but can be seen in some cases of
freeing this attachment at reoperation does not always
medial orbitotomy. Neither is the trochlea seen
cure the problem. It is best to avoid the complication
except for the cuff of tissue where the superior
by making sure that the inferior oblique is not includ-
oblique tendon exits. It is the 30 mm tendon of the
ed.
superior oblique that is dealt with during strabismus
Inferior rectus surgery. This tendon can be extremely variable in its
This muscle is very easy to engage on a hook site of insertion, tension, and even presence! Absence
and is usually found where it should be, 6.5 mm from of the superior oblique tendon, while not common, is
the limbus. Overlying the inferior rectus about 10 the most frequently noted absent extraocular muscle
mm behind the insertion is a thick mass of fascia (tendon). Most cases where the superior oblique ten-
which comprises Lockwood's ligament with the infe- don is encountered in surgery are cases of superior
rior oblique included. This tissue is also connected to oblique underaction or palsy. A superior oblique trac-
the lower lid retractors. In addition at one or some- tion test done before an incision is made will give
times both inferior rectus borders about 10 mm from strong evidence of a tendon anomaly. Since there is
the insertion are found large vortex veins lying on the chance of significant variation with the superior
sclera. These may be seen for several millimeters on oblique either contributing to underaction as in supe-
sclera before going through Tenon's capsule and into rior oblique palsy or rigidity as in Brown syndrome,
the orbit. The relationship of the inferior rectus with good exposure is required. For this reason it is a good
Lockwood's is especially important when dealing idea to consider a superior limbal incision to achieve
with thyroid ophthalmopathy patients requiring large a thorough look at the superior oblique tendon. An
inferior rectus recession. Some surgeons dissect even better idea is to do the cuffed superior limbal
intermuscular membrane well posterior to incision which is described on page 176. For success-
Lockwooods, even encountering fat, in order to ful superior oblique tendon surgery a good rule is to
achieve a large inferior rectus recession with minimal obtain good exposure and to expect the unexpected.
167
Chapter 6
168
Mechanics of surgery
D E
F G
Figure 4
A The site of the inferior cul-de-sac incision for approach- E The initial hook may be replaced with a Green hook that
ing the right medial rectus muscle has a prominent tip. Any muscle hook with a knob at the
B The conjunctiva is tented and scissors cut down through tip is suitable for this maneuver.
the conjunctiva, anterior, and posterior Tenons to bare F A second hook is placed in the incision but on top of the
sclera. muscle. It is moved back and forth to free the muscle in
C The initial incision can include only conjunctiva. With for- its capsule from overlying anterior Tenons capsule. This
ceps grasping anterior and posterior Tenons these tis- motion should extend several millimeters anterior to the
sues are tented up and excised. muscle insertion.
D A muscle hook enters the incision and with its tip slightly G The muscle hook that was used to separate the muscle
indenting sclera the hook passes beneath the rectus from anterior Tenons capsule is then used to pull the
muscle. conjunctival incision over the tip of the muscle hook that
is engaging the muscle.
continued.
169
Chapter 6
H I
J K
Figure 4, contd
H A small snip through intermuscular membrane exposes J Putting a second hook under the muscle provides better
the tip. exposure.
I With the muscle so exposed and after sufficient dissec- K The muscle is completely detached after curved locking
tion of the intermuscular membrane at the border of the forceps are placed on the stump, the first when half of
muscle, sutures are placed. If a resection is to be per- the muscle is freed and finally the second just before the
formed, a second hook under the muscle exposes suffi- last fibers are cut.
cient muscle tissue to allow placement of sutures for L The location of the incision when the eye and lids are in
resection. the physiologic state.
170
Mechanics of surgery
Limbal incision
The limbal incision in the conjunctiva for sur- the palpebral opening. If this incision is not closed
gical exposure of the rectus muscles is probably the very carefully with a smooth approximation of the
easiest to perform and the most versatile of the expo- conjunctiva - anterior Tenon's capsule layer with the
sure techniques for strabismus surgery. Wide expo- limbus, an unsightly ridge could result. This ridge
sure of the muscle and adjacent sclera with the inci- can also cause inefficient wetting of the peripheral
sion placed directly over the area of surgical activity cornea resulting in delle formation. The neophyte
makes suture placement in the muscle and needle strabismus surgeon should, in my opinion, learn the
placement in the sclera easier than with the cul-de-sac limbal incision first.
incision. When muscle transfer or insertion shift is The site of the limbal incision for medial rectus
done up or down, this wide exposure is especially surgery is centered at the insertion of the rectus mus-
beneficial. The relaxing incisions may be extended cle and extends 2 to 3 clock hour positions. The
10 mm or more without penalty because the addition- fusion of conjunctiva and anterior Tenon's capsule is
al length of these incisions is hidden behind the lids. grasped with fine-toothed forceps and tented up, and
The limbal incision also enables the surgeon to carry subanterior Tenon's capsule is entered with a No. 15
out conjunctival recession or conjunctival excision of Bard-Parker blade (Figure 5) scissors may also be
scarred tissue when necessary and also debulking of used as shown (Figure 5B). This blade incision is a
anterior Tenon's capsule in selected cases. A draw- puncture, not a dissection. The blade should not dig
back of this procedure is that an incision is made in into the episclera or sclera. Scissors should be used
A B
C
E
Figure 5
A The conjunctiva at the limbus is tented and a sharp blade C First, the radial incision is made,
passes beneath anterior Tenons capsule. D then the limbal incision,
B Scissors may be used to initiate the limbal incision. The E and then the second radial incision.
conjunctiva is tented and the scissor tips cut down along
the line of the radial incision through conjunctiva and
anterior Tenons capsule.
171
Chapter 6
to extend one relaxing incision. Scissors are then other border. Care should be exercised to avoid forc-
used to complete the limbal peritomy and then the ing the hook past or through an incompletely dissect-
second radial relaxing incision is made. These relax- ed plane. The intermuscular membrane is dissected
ing incisions are carried to but not through the plica from the borders of the muscle and the attachments
when the incision is made medially. Scissors and between the muscle sheath and the undersurface of
sharp dissection are used to sever the attachments anterior Tenon's capsule are dissected according to
between the muscle sheath and the undersurface of the surgeon's preference. Sutures are placed and the
anterior Tenon's capsule. Bare sclera is exposed at muscle is recessed as shown (Figure 7) or the intend-
one or both borders of the muscle's insertion by pierc- ed procedure is done. The limbal flap is closed at the
ing the intermuscular membrane at each edge of the apices with interrupted 8-0 absorbable sutures. An
muscle's insertion (Figure 6). It is imperative that additional suture may be placed in each of the radial
bare sclera be identified during this maneuver to incisions.
allow smooth passage of the muscle hook behind the The limbal flap may be recessed 5 mm using
insertion of the rectus muscle. Dissection of the mus- three sutures. Two of the sutures join the tips of the
cle capsule itself should be avoided and the muscle flap at the base of the relaxing wing incisions, and the
should remain in its capsule. Failure to maintain this third suture secures the center of the flap to the super-
technique produces unnecessary bleeding. ficial sclera or the muscle stump. The knot securing
When bare sclera is identified at each border of the conjunctival flap at the limbus may be buried by
the muscle, a muscle hook is passed easily behind the passing the needle from beneath the conjunctiva at
insertion. It is also acceptable to pass the muscle the limbus, and then from the conjunctival surface on
hook behind the insertion after exposing bare sclera at the flap, and finally tying the knot down tightly so
one border and cut down on the tip of the hook at the that it slides under conjunctiva.
B
C
Figure 6
A The conjunctiva-anterior Tenons flap is retracted and B A muscle hook is passed behind the muscle insertion.
scissors are used to penetrate posterior Tenons capsule C Shown here at surgery
exposing bare sclera at each muscle border.
172
Mechanics of surgery
B C
Figure 7
A After suture placement the muscle is recessed (or the C Shown here at surgery.
intended procedure is completed) and the surgical site is D The conjunctiva may be recessed.
observed. E The sutures may be placed to bury knots for more com-
B The incision is closed with fine absorbable suture joining fort in the immediate postoperative period.
the corners at the limbus.
173
Chapter 6
A B
C D
Figure 8
A Location of the conjunctival incision to expose the superi- C Location of the conjunctival incision to expose the inferior
or oblique medial to the superior rectus oblique
B Location of the conjunctival incision to expose the superi- D An enlarged limbal incision to expose both the lateral
or oblique tendon at its insertion rectus and inferior oblique
174
Mechanics of surgery
Figure 9
A The Barbie retractor C A wide Desmarres retractor retracting Tenons and con-
B A ribbon-maleable retractor elevating the superior junctiva over a horizontal rectus muscle
oblique tendon and superior rectus
175
Chapter 6
A B
C D
Figure 10
A The initial conjunctival incision is made temporally at the C The incision is carried out in the usual manner for the lim-
10 o'clock right eye and 2 o'clock meridian left eye 2 mm bal incision exposing the superior rectus and later the
posterior from the limbus. superior oblique tendon if that is the aim.
B The incision is continued through conjunctiva and anterior D The cuffed limbal incision is closed with several interrupt-
Tenon's for 4 or 5 clock hours leaving a 2 mm cuff on the ed absorbable 8-0 sutures with the knots buried.
limbal side.
176
7
Recession of a rectus muscle
Overview
Measured retroplacement or recession is the in vertical widening of the palpebral fissure.
standard technique for weakening a rectus muscle. Excessive recession of the superior rectus can cause
However, the term weakening or reducing the effect retraction of the upper lid and a widened palpebral
of a muscle may be a misuse of terms. Instead, retro- fissure. The unique relationship of the inferior rectus
placement of a rectus muscle provides a new starting to Lockwood's ligament and the inferior oblique
place or static alignment for the eye. In this new causes the inferior rectus, after recession, to be prone
position, the muscle's attachments to both anterior to both early and late posterior migration (slippage).
and posterior Tenon's capsule, the muscle pulleys, This results in undesirable overcorrection-hyper-
and adjacent structures continue to affect both static tropia, underaction of the muscle, and lower lid pto-
and dynamic factors in eye movement. The muscle's sis.
effect on the globe is mediated through these attach- To avoid this complication, the inferior rectus
ments as well as through the muscle's scleral inser- should be securely reattached to the globe. The prob-
tion. Unless the rectus muscle is recessed excessive- lem of lower lid ptosis after inferior rectus recession
ly, placing the new insertion behind the equator along can be reduced if not completely eliminated by taking
with extensive Tenon's dissection, the muscle's action two precautions. First, the intermuscular membrane
will not be compromised significantly in its field of (posterior Tenon's capsule) dissection should be car-
action. ried back 10 to 14 mm posterior from the inferior rec-
Both saccadic velocity and generated force will tus insertion. In most cases this maneuver requires
be the same after the usual recession. Excessive careful dissection to a point several millimeters pos-
recession, either by design as in special cases or as a terior to the entrance of the vortex veins. These are
complication, will result in decreased excursion of the found on one or both borders of the inferior rectus.
globe in the field of action of the muscle. These large These tortuous dark red veins, 1 mm in diameter, are
recessions can be used when treating patients with visible lying on sclera traveling for 5 mm or more
conditions such as; fibrosis syndrome, third nerve before piercing posterior Tenon's capsule and enter-
palsy, thyroid ophthalmopathy, nystagmus, or with a ing the orbit. Vortex veins bleed briskly if cut. Do
variety of complicated reoperations, and in the case of not cut or tear them! If this happens, pressure should
a slipped or lost muscle. Underaction occurs not be applied and then the veins cauterized after bleed-
because of any change in the contractile power of the ing has slowed. Second, the attachments of
muscle, but because of the alteration of mechanics of Lockwood's ligament to the inner surface of the infe-
the muscle-Tenon's-globe relationship. A muscle rior oblique should be dissected carefully to the same
inserting behind the equator of the globe will not level as the dissection of the intermuscular mem-
exert its full effect on globe rotation on a purely brane. All of this dissection is carried out without
mechanical basis. exposing fat. Another technique for avoiding lower
lid ptosis is to mark the relationship of Lockwood's
Excessive recession ligament to the inferior rectus before dissection and
In addition to underaction, excessive recession muscle recession and then to suture Lockwood's to
of the medial rectus will produce a widened medial the inferior rectus in the same relative position after
palpebral-canthal area. Excessive recession of the recession as it was before - effectively pulling the
inferior rectus causes ptosis of the lower lid resulting lower lid forward and upward.
177
Chapter 7
178
Recession of a rectus muscle
179
Chapter 7
180
Recession of a rectus muscle
Figure 4
After extensive dissection of the intermuscular membrane
the medial rectus, if it becomes detached from the globe,
can retract into the fat of the muscle cone resulting in a
lost muscle.
181
Chapter 7
182
Recession of a rectus muscle
A B
Figure 9
A Minimal superior rectus recession is 2.5 mm B Maximum superior rectus recession is not established. A
5.0 mm recession will place the new insertion anterior to
the superior oblique tendon.
would deflect the tendon posteriorly if this freeing guided just behind the superior rectus insertion. In
had not been done. spite of efforts to avoid this complication, the superi-
The superior rectus insertion can be engaged or oblique tendon may be inadvertently included with
from the medial or the lateral side (Figure 10). the hook which is intended to engage only the superi-
Careful dissection exposing bare sclera should be or rectus. If this inclusion goes unrecognized, the
completed before inserting the muscle hook. To superior oblique tendon may be reattached at the new
avoid engaging the superior oblique tendon, an inci- insertion of the recessed superior rectus (Figure 11).
sion is made to enter subposterior Tenon's space We have seen this complication at reoperation. If the
medial to the insertion of the superior rectus. The tip surgeon observes or suspects this unintended superior
of the muscle hook gently indents bare sclera and is oblique inclusion, a second muscle hook is passed
A B
Figure 10
A When identifying the entire superior rectus insertion it B The initial attempt to hook the medial rectus can be
may be necessary to pass a hook from either border and made from the medial side.
sometimes several times.
183
Chapter 7
A B
Figure 11
A Hooking the superior rectus from the nasal side can B A second hook introduced from the temporal side can
inadvertently include the superior oblique tendon. free the superior oblique tendon. The first hook is then
removed.
184
Recession of a rectus muscle
Figure 14
A A minimum inferior rectus recession of 2.5 mm
B A maximum inferior rectus recession of 5.0 mm. This
number is exceeded in cases of restriction and in special
circumstances.
Figure 16
The connections between the inferior rectus - inferior
oblique - Lockwoods ligament and the lower lid cause the
Figure 15 structures to move together.
Vortex veins are seen on both borders of the inferior rectus A Normal
when dissection is carried back 10 mm or more. B Lower lid ptosis after inferior rectus recession without
advancement of Lockwoods.
185
Chapter 7
border tends to remain constant. As the inferior rec- Barbie or other suitable retractor is used to provide
tus is recessed and Lockwood's ligament follows the adequate exposure. When the muscle is properly
muscle, the lower lid tends to drop lower producing exposed, it is stabilized with a muscle hook. The bor-
lower lid ptosis and widening of the palpebral fissure. der of the rectus muscle is elevated with a small hook
This problem can be avoided or at least reduced if to facilitate passage of the needle between the large
Lockwood's ligament is brought forward and sutured hook stabilizing the muscle and the small hook. The
to the inferior rectus so that it is the same distance needle passes through the tendon avoiding the anteri-
from the original insertion of the inferior rectus as or ciliary vessels. The anterior ciliary vessels should
before recession (Figure 17). Lockwood's ligament is not be severed by the needle but should be included
sutured to the surface of the inferior rectus with 6-0 in the suture (Figure 18).
absorbable suture at the same distance from the orig- After passing through the tendon, the needle is
inal insertion as it was preoperatively. brought again through the tendon including the ante-
rior ciliary vessels. The suture is then locked*
Rectus muscle recession (Figure 19). This technique for suture placement
technique
The standard technique for recession of each of
the rectus muscles is the same. Differences in maxi-
mum and minimum amounts and management of the
intermuscular membrane and check ligaments were
discussed previously. Because of the strength and
uniformity of synthetic absorbable suture, I prefer to A
recess a muscle using one double-arm suture.
However, two single-arm sutures may be used.
The rectus muscle is exposed by carefully
incising the intermuscular membrane and posterior
Tenon's capsule at 2 or 3 mm from the muscle border
with sharp dissection. Anterior Tenon's capsule aris-
es from the outer surface of the muscle sheath and is
outside of posterior Tenon's capsule. Anterior
Tenon's capsule is dissected with scissors from the
outer surface of the muscle until suitable exposure is
obtained. This varies according to each muscle. No
fat should be exposed. Large vessels bridging from B
the muscle capsule surface to the undersurface of
anterior Tenon's capsule may be simultaneously sev-
ered and sealed with cautery (see chapter 3). A
Figure 18
A Exposure of the rectus muscle insertion includes freeing
Figure 17 the insertion and proximal muscle borders sufficiently to
To assist in reducing lower lid ptosis when recessing the place the sutures.
inferior rectus the tissues around Lockwoods ligament are B A small hook elevates the muscle border to facilitate
pulled anteriorly and sutured to the inferior rectus at the placement of the needle through the muscle.
same distance from the inferior rectus insertion as in the C The needle is passed through the tendon avoiding the
preoperative state. anterior ciliary arteries.
* Mims has made specific recommendations for placing a secure locking bite, which is actually a true knot, at the rectus muscle border.
186
Recession of a rectus muscle
A B C
Figure 19
A If the sutures are passed as shown a true knot is C The muscle is detached.
formed.
B The anterior ciliary arteries are ligated the hook is aimed
back toward the orbit (not shown).
behind the first hook reduces the likelihood of inad- measuring medial rectus recessions and continue to
vertently cutting the suture when detaching the mus- use it. When comparing measurement from the lim-
cle and reduces bleeding by ligating the anterior cil- bus with measuring from the insertion, the distance
iary vessels. The resection effect is negligible in my between the insertion and the limbus should be added
opinion. After the sutures have been pulled posteri- to the intended amount of recession. When measured
orly from the insertion, the muscle is cut off flush from the limbus, a traditional 4 mm recession of the
with the sclera using scissors and taking small snips. medial rectus would indicate that the new insertion
Several footplates may need to be severed before the should be 9.5 mm from the limbus, assuming that the
muscle retracts freely. The muscle hook is now medial rectus insertion is 5.5 mm from the limbus.
behind the suture line for cutting the muscle free, Clinical experience has shown that the average medi-
whereas it was in front of or closer to the insertion al rectus insertion in esotropia is 4.4 mm with a range
when the sutures were placed. of 3 to 6 mm. Measuring from the limbus opposite
A caliper or scleral ruler is used to measure the the corner of the insertion advances the muscle a bit
amount of recession. Measurements may be taken (decreasing the recession) and measuring a chord pro-
from the original insertion or the limbus. I prefer to duces excess recession. These factors are offsetting
measure most medial rectus recessions from the lim- (Figure 20).
bus. The surgeon should decide on one method of
B C
Figure 20
A A caliper measures from the limbus or the original inser- C The scleral ruler measures the true distance over the
tion. scleral surface while calipers measure the chord.
B A scleral ruler modified from William Scott is also useful
for this measurement.
187
Chapter 7
B C
Figure 21
A A passage of the needle through sclera. C The recessed muscle is ideally parallel to the old inser-
B The scleral tunnel should be 1.5 mm or more. tion (or nearly so).
The tip of the caliper or scleral ruler may be lized with a utility forceps, which may be of a lock-
used to make a dimple in the sclera. This provides a ing variety, grasping the insertion stump opposite the
point indicated by a blue mark which is the uvea site of needle placement. Care should be taken to
showing through the sclera. This dimple can be used space the two suture bites in the sclera approximately
as a means of engaging scleral tissue with the needle 10 mm apart and equidistant from the limbus or inser-
tip. The thin, spatulated needle displaces tissue and tion. This maneuver ensures that the new insertion
should be in the superficial one-third to one-half of will be parallel with the limbus, smooth, and flat. The
the scleral thickness. During experiments in the lab- sutures are tied with a surgeon's knot, and a total of
oratory, Coats and Paysse found that a scleral bite 1.5 three throws are taken. The knots should be tied care-
mm long and .2 mm deep is sufficient to secure the fully, using smooth-tying forceps, grasping the suture
muscle to sclera. This bite had a pull out strength in very close to the knot.* This approach ensures secure
excess of 200 gm. This is greater than the physiolog- knots and reduces the possibility of a broken suture.
ic muscle force exerted on this reattachment. A
longer, but not deeper scleral bite can be taken to pro- Variations in suture and
duce friction in the scleral suture tunnel. This holds needle placement
the muscle in place during suture tying. A wide variety of techniques may be used to
The needle is inserted at the caliper mark secure the suture to the tendon and then to reattach the
(Figure 21). A scleral bite of 1.5 mm or more is tendon to sclera. The overriding principles are secu-
taken, but care should be exercised to ensure that the rity, consistency, and safety. The suture must be
needle is always in sight through the scleral lamellae securely attached to the tendon to avoid slippage of
and does not perforate the sclera. The globe is stabi- the tendon-muscle. The muscle and tendon must be
* Some surgeons with smaller hands than mine prefer to tie sutures using their fingers rather than forceps.
188
Recession of a rectus muscle
securely attached to sclera to avoid pulling free from placed. The scleral bite is taken and the suture is tied.
sclera and producing muscle slippage. Finally, the The crossed swords technique of Parks may also be
needle track in sclera must be deep enough and long used. The first needle is left in the long scleral tunnel
enough to secure the tendon-muscle to sclera without which angles toward the insertion while the second
going too deeply and entering the eye by passing needle is placed in the sclera, crossing the first. Both
through choroid and retina into the vitreous. needles are advanced carefully pulling the first suture
As a variation of the double-arm suture tech- through only when the round part of the other needle
nique, a single suture may be used, taking a bite into remains in the track. This maneuver prevents the sec-
the central tendon and then tied. Additional bites then ond needle from cutting the first suture while in its
are taken at the muscle borders and locked loops are scleral track (Figure 22).
Figure 22
A A bite secured with a knot can be taken at mid-muscle B The properly recessed muscle with a double arm suture.
and sutures brought out the borders and locked for C Needles can be placed in the crossed swords technique
added security. producing a longer scleral tunnel. This holds the muscle
in place while the suture is being tied.
189
Chapter 7
Figure 23
A For treatment of A and V the medial recti are shifted to B The medial recti are recessed and shifted up to treat an
the closed end and the lateral recti are shifted to the A esotropia.
open end. This assumes that the proper horizontal sur-
gery has been done. continued.
190
Recession of a rectus muscle
191
Chapter 7
A B
C D
Figure 24
A 1 mm resection effect with a posterior (safe) suture D With no (or minimal) resection effect the same point of
placement. reattachment is achieved, but the muscle is a bit longer
B With a small resection effect, the new point of insertion is or more redundant.
more important than the slight reduction in the redundant E A potential pitfall of reducing the resection effect by
muscle. placing the suture as near as possible to the distal end of
C If the suture securing the muscle before it is detached the muscle before detaching it from the globe is slippage
from the globe is placed distal to the muscle hook (closer of the tendon and muscle in the capsule. This
to the insertion), the resection effect is avoided or at complication, called a slipped muscle is responsible for
least diminished. some early and late overcorrections, particularly after
medial rectus recession for infantile esotropia.
192
Recession of a rectus muscle
Hang-back recession
The hang-back recession has been described as with the hang-back technique. In humans who had
"a simple, safe alternative to conventional recession." hang-back recession of the superior rectus, reattach-
The procedure is said to be less likely to result in scle- ment 11.5 and 12.0 mm from the insertion (as intend-
ral perforation because needles are placed through ed by the surgeon) was confirmed by x-ray study of a
relatively thicker sclera near the insertion site. stainless steel suture placed at the end of the superior
Another reported safety factor is that because the rectus. Hang-back recession of the inferior rectus is
suture placement site is more anterior, it is more likely to result in the complication of lower lid ptosis
accessible to the surgeon. Results are said to be com- and muscle slippage. The inferior rectus muscle may
parable to conventional recession when appropriate be least suitable for hang-back recession, except in
doses are used. The attachment site has been shown desperate cases as might occur in some cases of fibro-
to be about where it was intended on studies carried sis syndrome and severe thyroid ophthalmopathy
out on monkeys who had horizontal recti recessed (Figure 25).
A B
C D
Figure 25
A Hang-back recession of the lateral rectus muscle through C Sutures may be brought through the muscle stump at the
a cul-de-sac incision. 6-0 synthetic absorbable suture is width of the muscle. A limbal incision is used.
used. D The amount of hang back recession is measured for the
B The knot in the suture is pulled to bring the cut end of superior rectus.
the muscle to the muscle stump. The suture is
measured with calipers to determine the distance the
muscle is recessed from the insertion.
193
Chapter 7
Figure 26
A For head tilt to the left, the nasal half of the right superior B For head tilt to the right, the temporal half of the right
rectus and the temporal half of the left superior rectus is superior rectus and the nasal half of the left superior
recessed and this procedure is continued around the rectus is recessed and this procedure is continued
globe to rotate the eye in the direction of the head tilt. around the globe.
194
Recession of a rectus muscle
Figure 27
A The anterior ciliary artery is dissected from the muscle C Sutures are placed in sclera at the point of intended
substance and is lifted on a small hook or cannula. recession.
B Sutures are placed at the borders of the muscle while
the arteries are lifted. continued.
195
Chapter 7
D E
Slanted recession
Slanted reattachment of a recessed horizon-
tal rectus muscle suggested by Nemet has been used
for treatment of A and V. The muscle edge that is A
to be weakened more is recessed farther back com-
pared to the other edge. This follows the principle
of selective weakening by moving the entire muscle
in the direction that you intend to weaken the mus-
cle (Figure 28).
Figure 28
A Medial rectus top edge recessed 5 mm farther for treat-
ing an A esotropia.
B Medial rectus recession with the lower edge recessed 5
mm farther back to treat a V esotropia.
196
Recession of a rectus muscle
197
8
Resection of a rectus muscle
Overview
Resection of an extraocular muscle is general- more often than we had suspected. In a recent series
ly classified as a strengthening procedure. But of patients undergoing reoperation of the lateral rec-
removal of all or part of a muscle's tendon with or tus for both overcorrection and undercorrection and
without inclusion of some muscle fibers merely short- after resection or recession, 38% of patients had the
ens and does not actively strengthen a muscle, at least inferior oblique included at the inferior border of the
after the initial reflex spasticity subsides. The princi- lateral rectus. Of course, this series studied only
pal benefit of a resection may be to enhance the effect those patients needing reoperation; nonetheless, this
of a recession procedure done on the antagonist mus- specific surgical complication is one we encounter
cle. Actually, if muscle fibers are removed at the time frequently in our practice.
of resection, theoretically the muscle should be weak- As an alternative to rectus muscle resection,
er! In the clinical setting, this does not seem to be the there has been a revival of the tucking procedure for
case. As I became more aware of the relationship these muscles. An advantage of tucking is that the
between passive mechanical factors and dynamic anterior ciliary circulation of the tucked muscle can
neural factors in the surgical management of strabis- remain patent. I have used this technique on the last
mus, I performed more recessions and fewer resec- rectus muscle in a patient who had had the other three
tions. rectus muscles detached.
Although resection procedures are relatively
easy to perform, they can result in more redness and Horizontal rectus resection
lumpiness of the conjunctiva, particularly in the area (medial and lateral rectus
of the medial rectus. Natural barriers to orbital fat are muscles)
also brought more anteriorly around the medial rec- The minimum amount of resection of either a
tus, promoting the possibility of unsightly fullness medial rectus or a lateral rectus muscle is 5 mm,
after resection. Nevertheless, resection of a rectus regardless of the age of the patient (Figure 1). In gen-
muscle is indicated in many strabismus cases. eral, a resection of a horizontal rectus muscle is less
In addition to conjunctival problems that occur effective in altering ocular alignment than a recession
after resection of the medial rectus, palpebral fissure of the same amount; hence the larger relative minimal
narrowing can occur after inferior rectus resection, values for horizontal rectus resection.
and some ptosis of the upper lid, which is manifested The maximum resection for a horizontal rectus
by narrowing of the palpebral fissure, after superior muscle is 8 mm for infants less than 1 year and ordi-
rectus resection. narily 10+ mm for older children and adults.
The inferior oblique may be included inadver- However, upper limit figures for resection procedures
tently during resection of the lateral rectus. This are very loosely adhered to, in contrast to the mini-
avoidable complication can cause limited elevation mum figures which tend to be strictly followed. In a
and/or depression in the involved eye, often with patient with a very large deviation and a reason to
mechanical restriction causing unexpected horizontal limit surgery to one eye, horizontal rectus resection of
and vertical strabismus. This complication occurs
199
Chapter 8
Figure 1
A Minimum dissection of a horizontal rectus muscle is 5 B Maximum resection of a horizontal rectus is 10 mm (in
mm. special cases this can be increased to 14 mm.
200
Resection of a rectus muscle
A B
C D
E F
Figure 2
A The rectus muscle in its capsule with intermuscular E Or, the muscle clamp is placed after intermuscular mem-
membrane intact. brane is dissected several mm posterior to the extent of
B Intermuscular membrane is dissected to the extent of the resection.
resection. F The new insertion is free of intermuscular membrane. Be
C The resection clamp is placed - sutures are placed sure the muscle is firmly attached to avoid a possible
according to the surgeons preference. lost muscle!
D After resection the intermuscular membrane is at the
level of the new insertion.
201
Chapter 8
Suture placement
After the muscle clamp has been placed accord-
ing to the measured amount of the intended resection,
the tendon is severed from its insertion. A 1 mm
tendinous stump should be left at the insertion to
serve as an anchoring place for sutures. The sclera B
immediately behind the insertion of the rectus mus-
cles is only 0.3 mm thick so this tendinous stump pro-
vides a safety factor during suture placement (Figure
3).
Double-arm sutures are inserted in a backhand
manner through the tendinous insertion. At this time
care should be taken to free the under surface and
inferior border of the lateral rectus from the inferior
oblique. If this is not accomplished, the inferior
oblique could be brought forward to the new insertion
of the resected lateral rectus, causing inferior oblique
inclusion which produces a postoperative vertical and
sometimes horizontal deviation.
The sutures are carried through the muscle
behind or posterior to the resection clamp. The assis-
tant grasps the needle tip and pulls the suture through.
The two double-arm sutures are placed in a horizon- C
tal mattress fashion, first through the insertion, and
then through the muscle behind the clamp (Figure 4).
After the sutures have been placed through the
muscle, the resection clamp is loosened and moved to
the tip of the tendon. A Nugent or other suitable for-
ceps is used to hold the tip of the tendon while the
resection clamp is advanced. Traction is placed on
the muscle clamp to advance the muscle so that the
point of passage of the sutures through the muscle is Figure 3
directly over the line of the original muscle insertion. A Measuring the resection
B Cutting the muscle from the globe. Leave a 1 mm
The sutures are tied securely with a surgeon's knot. A stump.
hemostat is used to crush the tendon just anterior to C A suture is placed backhand through the stump.
the point where the sutures are tied. A battery-oper-
ated cautery is used at this point to cauterize the
crush line on the muscle to control bleeding before
cutting off the muscle-tendon to be resected. Scissors
are used to excise the excess tendon (Figure 5).
202
Resection of a rectus muscle
A B
Figure 4
A The needle passes through the muscle behind the B The process is continued with two double armed mat-
clamp. tress sutures in place.
A B
C D
Figure 5
A The muscle clamp is advanced to the end of the muscle. C The muscle is crimped just anterior to the sutures with a
B The sutures are tied securely as the resection line is hemostat.
brought over the stump. D The crimped line is cauterized.
E The excess muscle tissue is excised.
203
Chapter 8
With the excess tendon removed, the shortened producing a smooth appearance to the conjunctiva
muscle abuts the point of the original tendinous inser- overlying the resected muscle insertion. Sutures may
tion. The double horizontal mattress sutures with be placed through the stump of the muscle from the
bites several millimeters apart afford a secure union muscle side. A lap joint is produced. It is also possi-
of the resected muscle across its entire width. A cross ble to put the needles through the muscle first and
section at the point of union shows that the tendon then through the insertion producing a butt joint
stump and muscle are joined in a slightly puckered (Figure 6).
butt joint. This gradually settles over several weeks,
Figure 6
A A butt joint. C Sutures brought through the muscle and then through
B A lap joint. the stump produce a butt joint.
204
Resection of a rectus muscle
B C
D E
Figure 7
A A single double arm suture is placed suitable for the C The sutures are tied.
intended resection. Locking bites are then placed imme- D For added security the sutures can be brought back
diately behind the suture. through the stump and muscle...
B After cutting out the section of muscle to be resected, the E ...and tied securely.
sutures are brought through the stump.
205
Chapter 8
A B
C D
Figure 8
A Two single arm sutures are placed at the muscle border C The muscle is cut anterior to the sutures.
and are secured with a square knot. D The resected muscle is cut leaving a 1 mm stump.
B The muscle is crimped. continued.
206
Resection of a rectus muscle
E F
Figure 8, contd
E The sutures are tied F An optional third suture is placed at the middle of the
insertion to close any gap.
A B
Figure 9
A A second hook exposes the inferior rectus back to B Two double arm sutures are placed at the muscle bor-
Lockwoods ligament. ders, a second loop is added and secured with a knot.
continued.
207
Chapter 8
C D
Figure 9, contd
C The muscle is crimped anterior to the sutures. E The muscle is sutured to the globe with a central suture
D The muscle is cut in front of the sutures and then at the added.
insertion leaving a 1 mm stump.
208
Resection of a rectus muscle
B C
E
D
Figure 10
A Two double arm sutures are placed about 1 mm apart C The muscle is crimped anterior to the suture.
with an added loop secured with a knot. D Cautery is placed on the crimped line
B The anterior suture is at the point of intended resection. E The muscle is cut.
continued.
209
Chapter 8
F G
210
Resection of a rectus muscle
A B
C D
Figure 11
A A double arm suture is placed at each muscle border at C The sutures are tied creating a loop.
the point equal to the intended muscle shortening and D The tip of the loop is secured to the top of the muscle.
are secured with a knot.
B The sutures are brought through the insertion of the
muscle.
Displacement of horizontal
rectus muscles with resection
for A and V patterns
As with all vertical displacements of the hori- and the lateral rectus muscles weakened for exodevi-
zontal rectus for treatment of vertically incomitant ations.
strabismus, the lateral rectus muscles are moved A recession-resection procedure is carried out
toward the open end of the pattern, and the medial on the left eye of a patient with a V pattern exotropia.
rectus muscles are moved toward the closed end of The resected left medial rectus is shifted one-half
the pattern. The surgeon needs to remember that muscle width downward, and the recessed left lateral
medial rectus muscles are weakened and the lateral rectus is shifted one-half muscle width upward
rectus muscles strengthened for esodeviations; con- (Figure 12).
versely, the medial rectus muscles are strengthened
211
Chapter 8
Figure 12
A The medial rectus is resected and moved down while the B The resected medial rectus is moved up and the
lateral rectus is recessed and moved up to treat a V XT. recessed lateral rectus is moved down for an A XT.
Figure 13
A The eyes are ET 40D with a right hypotropia of 15D.
continued.
212
Resection of a rectus muscle
213
9
Surgery of the obliques
215
Chapter 9
elongation of the tendon? For congenital superior A half century ago, the superior oblique was
oblique palsy with a lax or misdirected tendon, I do a described as nolo tangere or do not touch. This
strengthening or shortening procedure of the superior advice is no longer valid, but I would replace this
oblique tendon. If the tendon is absent, the antago- advice with the recommendation for superior oblique
nist, or yoke muscle, is weakened and, in some cases, surgery, handle with care. In contrast, the inferior
the ipsilateral superior rectus is weakened. oblique is weakened frequently and in most cases
A newer oblique muscle procedure is the ante- results are good. While the superior and inferior are
rior transposition of the inferior oblique. This proce- both oblique muscles, the similarity ends there.
dure is performed in cases of overaction of the inferi-
or obliques with V pattern and dissociated vertical Weakening the inferior oblique
deviation. The new insertion of the inferior oblique is
placed just anterior to the temporal corner of the infe- Inferior oblique myectomy
rior rectus insertion. In addition to weakening inferi- When performed carefully, an inferior oblique
or oblique action, it seems to have a tethering effect myectomy can be completed with little or no bleeding
on the upward movement of the globe and therefore and fat should never be encountered. Because the
lessens the amplitude of vertical deviation of the inferior temporal vortex vein may be encountered, it
DVD. must be dealt with carefully to avoid rupture. If the
Stager has devised a procedure placing the new vein is severed, copious bleeding will occur. This
insertion of the inferior oblique nasal to the inferior would be controlled with tamponade and/or cautery.
rectus. This changes the inferior oblique from an Given the more or less blind sweep of the hook it is a
extorter to an intorter and is therefore potentially use- pleasant and certainly welcome occurrence that vor-
ful in cases with large extorsion. tex vein rupture in this area occurs so infrequently. In
Other surgical procedures of the obliques nearly 40 years, I have not seen this.
include weakening procedures of the superior oblique Persistent inferior oblique overaction can result
usually performed by means of tenectomy, tenotomy, if a slip of muscle is not cut or if the proximal cut end
or disinsertion. Marginal tenotomy of the superior of the inferior oblique attaches to the sclera resulting
oblique has been described, but it is not a logical in scarring and fibrosis. This is avoided by tucking
choice because it is either not effective or it becomes the proximal end of the inferior oblique behind poste-
a complete tenotomy because of the cable-like make- rior Tenon's capsule.
up of the tendon. A few surgeons prefer to weaken The incision for exposure of the inferior
the superior oblique by recession. No clear-cut dif- oblique muscle is approximately 8 mm long. It is
ference in results of these weakening procedures has located 8 mm from the limbus and is concentric with
been provided. The complex anatomy of the superior it . It is also anterior to the inferior fat pad (Figure 1).
oblique tendon as it relates to the superior rectus and The eye is stabilized in elevation and adduction with
superior orbital fascia contributes to the differing a locking forceps. One or two fine-toothed forceps
results from the various weakening procedures. are used initially to elevate the conjunctiva, Tenon's
Transfer of the superior oblique tendon, with- capsule, and intermuscular membrane, and a snip
out fracture of the trochlea, is accomplished in some incision is made between the forceps, exposing bare
cases of third nerve palsy. Fracture of the trochlea to sclera (Figure 2). Blunt-tipped Wescott scissors are
achieve removal of the tendon is, in my opinion, not inserted into the incision against bare sclera and the
practical and should not be attempted. Shift of the scissors tips are spread to separate with blunt dissec-
anterior insertion fibers (or the entire insertion) is per- tion the filamentous attachments between the sclera
formed to enhance intorsion in selected cases of supe- and posterior Tenon's capsule.
rior oblique palsy, especially in bilaterally involved To expose the anterior border of the inferior
individuals with torsional diplopia. oblique muscle, the surgeon first places a large mus-
A technique for treating Brown syndrome cle hook behind the insertion of the lateral and the
employs a band of silicone used as an expander to inferior rectus muscles. Then a third muscle hook is
lengthen the superior oblique tendon nasal to the used to elevate the posterior border of the conjuncti-
superior rectus. A variety of procedures for weaken- va - Tenon's capsule incision. Deep in the incision at
ing the superior oblique in cases of Brown syndrome the junction of the sclera and posterior Tenon's cap-
are done, ranging from disinsertion of the posterior sule, the anterior border of the inferior oblique will be
seven-eighths of the insertion to tenectomy near the seen.
trochlea. However, any technique can fail in some A small hook is placed flat against sclera,
cases and succeed in others. This tells us that no sur- indenting it slightly with the tip toward the inferior
gical procedure or strabismus surgeon (at least that I rectus (or toward the lateral rectus). The hook is gen-
have heard of) has the surgical answer for all cases of tly slid beneath the inferior oblique muscle until the
Brown syndrome. orbital wall is felt. The tip of the hook is then rotated
216
Surgery of the obliques
A B
Figure 1
A The site of the incision for exposing the inferior oblique. B A snip incision going through conjunctiva, anterior
Tenons capsule, and intermuscular membrane exposes
bare sclera.
A B
C D
Figure 2
A Blunt dissection frees intermuscular membrane from C A small hook slides posteriorly along sclera and is rotat-
sclera. ed when it is behind the posterior border of the inferior
B Large muscle hooks are placed behind the insertion of oblique.
the lateral and inferior rectus and a small hook lifts inter- D The small hook brings the inferior oblique forward.
muscular membrane exposing the anterior border of the
inferior oblique.
217
Chapter 9
until it points at the junction of the lateral and inferi- is included and avoiding making a hole in the inter-
or orbital rim. As the inferior oblique is engaged, the muscular membrane (posterior Tenon's capsule)
hook retracting conjunctiva is pulled back to expose which would result in prolapse of orbital fat (Figure
the tip of the small hook that shows through Tenon's 3). Fortunately, the inferior temporal vortex vein is
fascia and is beyond the posterior muscle border. very infrequently ruptured. However, excess blind
When the inferior oblique muscle is engaged on the manipulation in this area should be avoided to lessen
teaser hook, the surgeon must take great care to bring the chances of this annoying complication.
the muscle forward, making sure that only the muscle
A B
C D
Figure 3
A The tip of the small hook is seen behind the posterior D The hooks, or larger hooks that have replaced the small
border of the inferior oblique. hooks, are rotated to inspect the inferior oblique poste-
B A knife or scissor exposes the tip of the hook. rior edge of the inferior oblique with the under surface of
C A second hook is introduced. posterior Tenons capsule behind.
E If some inferior oblique muscle is seen behind the hooks,
it is carefully included by placing a second pair of hooks.
218
Surgery of the obliques
A scissors or a scalpel blade is used to cut indicates that the entire inferior oblique has been
down on the tip of the small hook, exposing it behind engaged. At this point a vortex vein will be seen leav-
the inferior oblique. A second hook is placed and the ing sclera and passing through intermusuclar mem-
fascial layers associated with the muscle are dissect- brane. If the inferior oblique has been hooked incom-
ed from the muscle, exposing 5 to 8 mm of the infe- pletely, a red stripe will be seen horizontally below
rior oblique. The muscle hooks behind the insertions the hooks. This strip of muscle should be picked up
of the lateral and inferior rectus muscles may be gently with two small hooks, repeating the procedure
removed as soon as the second hook is placed under until the entire inferior oblique muscle has been
the inferior oblique. The small hooks under the infe- engaged. Two hemostats placed 5 to 8 mm apart are
rior oblique are replaced by two larger hooks, which used to clamp the inferior oblique muscle belly. With
are rotated away from the scleral surface and the scissors or a scalpel blade, a 5 to 8 mm segment of the
undersurface of the inferior oblique. At this time it inferior oblique muscle belly lying between the
can be determined whether the entire inferior oblique hemostats is excised. Cautery is then applied heavily
has been engaged. A clearly defined border of the to each cut end for hemostasis (Figure 4).
inferior oblique with white Tenon's capsule below
A B
C D E
Figure 4
A Hemostats are placed with a 5-8 mm segment of muscle D The small hole in posterior Tenons capsule can be
between. closed with an 8-0 absorbable suture.
B After the segment of muscle has been cut out with scis- E Conjunctiva is closed with an 8-0 absorbable suture.
sors or a scalpel, the cut ends are cauterized.
C The inferior oblique is allowed to retract or the inferior
oblique may be nudged into the hole in Tenons.
219
Chapter 9
After the hemostats are removed, the inferior Alternative weakening procedures of
oblique muscle is allowed to retract and the conjunc- the inferior oblique: recession and
tival incision is closed with either interrupted or run- disinsertion
ning sutures, or this incision may be left unsutured,
depending on the surgeon's preference. If the proxi- Two other techniques for weakening an over-
mal cut end of the inferior oblique fails to retract into acting inferior oblique are recession and disinsertion.
the space outside posterior Tenon's capsule, it can be Recession of the inferior oblique can be graded and is
nudged into the space with the tip of a muscle hook especially useful in cases where a minimal amount of
or forceps. As the inferior oblique retracts into the weakening is required. Because the new inferior
defect in Tenon's capsule and approaches the lateral oblique insertion is placed at a specific point on the
border of the inferior rectus, a small slit in posterior globe after recession, finding the inferior oblique at a
Tenon's capsule can be seen. This slit can be closed subsequent operation is easier to accomplish than
with one or two 8-0 Vicryl sutures. after myectomy or disinsertion.
The incision, localization, and exposure for
A common complication of inferior recession or disinsertion of the inferior oblique mus-
oblique weakening and how to avoid cle are the same as described previously for a myec-
it tomy. Recession of the inferior oblique muscle is
begun by placing either two single-arm sutures or a
As is the case with any of the extraocular mus- single double-arm suture through the inferior oblique
cles, the function of the inferior oblique depends on muscle near the lower border of the lateral rectus.
its having some tissue connecting the origin and (Figure 5). The suture is therefore placed approxi-
insertion. Contraction of the muscle causes these two mately a millimeter from the muscle's broad inser-
points to be brought closer together. The muscle's tion. To ensure inclusion of all of the muscle fibers at
effect is manifested through movement of the globe this point, careful inspection of the posterior border of
toward the fixed point or origin of the muscle. the inferior oblique insertion should be carried out.
If in the process of performing a myectomy or The surgeon must detach the entire width of the infe-
any weakening procedure of the inferior oblique mus- rior oblique muscle, freeing the muscle completely
cle the clamps or recession sutures exclude a portion from the sclera for the recession to be effective.
of the muscle, a band of uninterrupted muscle tissue The inferior oblique is reattached to the sclera
with associated intermuscular membrane remains at a point that depends on the amount of recession
connecting origin and insertion. A portion of the infe- intended. Fink described an instrument for locating
rior oblique coursing uninterrupted between origin the point of reinsertion (see page 12), but recession is
and insertion acts somewhat like a tendon. Inferior now usually accomplished by reattaching the inferior
oblique weakening would then be less than expected. oblique in relation to existing landmarks. For exam-
This complication which causes undercorrec- ple, Parks reattached the anterior corner of the inferi-
tions can be avoided. Careful inspection of the pos- or oblique 2 mm lateral and 3 mm posterior to the lat-
terior aspect of the inferior oblique muscle reveals eral border of the inferior rectus insertion. The poste-
any remaining bands. These bands are engaged on rior scleral reattachment is placed according to the
muscle hooks and a myectomy is repeated on this width of the inferior oblique muscle. Other tech-
smaller segment of the inferior oblique muscle. For niques reinsert the inferior oblique slightly more pos-
an inferior oblique myectomy to be effective, a seg- teriorly. Regardless of the intended amount of reces-
ment of inferior oblique that includes its entire width sion, when the line of pull of the inferior oblique is
must be removed. A partial myotomy of the inferior maintained the new effective insertion is at the later-
oblique in my experience is ineffective. When disin- al border of the inferior rectus. Changing the line of
sertion of the inferior oblique is chosen for weaken- pull of the inferior oblique, as in anterior transposi-
ing this muscle, care must be taken to sever the entire tion, both weakens the pull of the inferior oblique
insertion. Some surgeons perform marginal myoto- and tethers or mechanically limits elevation.
my of the inferior oblique and claim good results. I do Another technique for weakening the inferior
not recommend this procedure. oblique is disinsertion. In this procedure, after iden-
tifying and hooking the inferior oblique in the usual
manner, the insertion of the inferior oblique is
exposed while the lateral rectus is elevated on two
muscle hooks. The inferior oblique is detached from
the sclera. The muscle is allowed to retract and the
incision is closed (Figure 6).
220
Surgery of the obliques
A B
Figure 5
A For recession, after exposing the inferior oblique, the lat- B The muscle is cut from the globe.
eral rectus is lifted and one or two sutures are placed in C The muscle is reattached along the line of pull approxi-
the inferior oblique one or two millimeters from the inser- mately 6 to 8 mm from the insertion.
tion.
A B
Figure 6
A For disinsertion, after exposing the inferior oblique in the B The inferior oblique is cut at its insertion and is allowed
usual way, the lateral rectus is elevated on two hooks to retract.
exposing the inferior oblique insertion.
221
Chapter 9
222
Surgery of the obliques
A B
C D
Figure 8
A The insertion is exposed. C The muscle is detached and from here disinsertion,
B The muscle is clamped near the insertion. recession, myectomy or anterior transposition can be done.
D conjunctiva is closed.
oblique. It is unlikely, in my opinion, that the inferi- just temporal and anterior to the inferior rectus inser-
or oblique is actually converted to a depressor. tion. The tip of the inferior oblique is sutured to scle-
The indication for anterior transposition of the ra 1 or 2 mm anterior to the lateral border of the infe-
inferior oblique is overaction of the inferior obliques rior rectus insertion, immediately adjacent to the infe-
causing excess elevation in adduction and a V pat- rior rectus.* The new inferior oblique insertion
tern plus dissociated vertical deviation. This proce- should be just anterior but parallel to the inferior rec-
dure should not ordinarily be performed on just one tus insertion. The conjunctiva may be closed with
eye because of the possibility of producing a large one or two 8-0 Vicryl sutures. A mound of inferior
secondary deviation. I have done it in one patient, but oblique muscle, obvious just behind the limbus
soon after this procedure did it in the other eye. immediately after surgery, subsides in a few weeks
To perform anterior transposition, the inferior and does not present a problem. But, fullness of the
oblique muscle is isolated and a 6-0 Vicryl suture is lower lid persists in some cases.
placed at the distal end of the inferior oblique (Figure
9). A large hook is then placed to expose the sclera
*Surgeons differ in their choice for positioning the new insertion of the inferior oblique, placing it even with the inferior rectus or a mil-
limeter behind.
223
Chapter 9
A B
D
C
Figure 9
A A suture is placed at the distal inferior oblique and the D The level of attachment of the inferior oblique can vary
muscle is detached. from a few millimeters ahead of to a few millimeters
B A hook is placed behind the inferior rectus insertion... behind the level of the inferior rectus insertion.
C ...and the inferior oblique is secured to sclera. E The conjunctiva is closed.
224
Surgery of the obliques
A B
Figure 10
A A tucker is placed. B The loop is 5 mm on each side of the tucker (10 mm
total). It is secured with 5-0 non-absorbable suture.
A B
Figure 11
A Sutures are placed at the borders of the inferior oblique. B The inferior oblique is detached and reattached at the
upper border of the lateral rectus.
225
Chapter 9
226
Surgery of the obliques
A B
More
C D weakening
Less
weakening
F
E
Figure 13
A The tip of the hook behind the tendon and fascia is D The location of the cut influences the amount of weaken-
exposed with a scissors or scalpel blade ing.
B The fascia is carefully separated from the tendon fibers E The tendon retracts.
and a hook is placed behind the isolated tendon. F The conjunctiva is closed.
C A second hook is placed behind the tendon and the ten-
don is cut.
227
Chapter 9
228
Surgery of the obliques
B C
D E
F G
229
Chapter 9
7/8 tenotomy/disinsertion
After exposing the insertion of the superior from its insertion, and a surgeon's knot is tied. This is
oblique tendon, scissors are used to disinsert the ten- to ensure that the suture is placed in solid tendon. The
don or to carry out a 7/8 posterior tenotomy of the tendon is then transected between the suture and the
superior oblique tendon with excision of a triangle tendon's insertion. The tendon is allowed to retract
shape portion of the tendon at the insertion (Figure beneath the superior rectus for a distance of 8 to 20
15). mm, according to the intended amount of recession,
and the suture is tied in a hang loose fashion at the
Recession of the superior oblique tendon insertion (Figure 16).
For a more controlled, weakening of the supe-
rior oblique tendon, recession in the place of tenoto-
my can be done. Split tendon lengthening of the
superior oblique tendon nasal to the superior rectus
has been described and is performed by some sur-
geons in selected cases. It has also been performed
and abandoned by other surgeons. While this tech-
nique is theoretically possible, it is difficult to per- A
form. I see no reason to use it in place of other avail-
able superior oblique weakening procedures and for
that reason it will not be illustrated.
In preparation for recession, the superior
oblique tendon is located and engaged at its insertion
temporal to the lateral border of the superior rectus.
A double-arm 5-0 Mersilene or 6-0 Vicryl suture is
placed through the superior oblique tendon 4 mm
B
A
Figure 16
A The superior oblique tendon is exposed at the insertion
temporal to the superior rectus.
B A double arm suture is placed 4 mm from the insertion
Figure 15
and the tendon is cut free of sclera at the insertion.
Disinsertion - 7/8 tenotomy C The needles are brought through the insertion. After the
A Posterior 7/8 tenotomy tendon is allowed to retract a graded amount according
B Disinsertion to the amount of weakening needed, the suture is tied.
230
Surgery of the obliques
A B
C D
Figure 17
A The superior oblique inserts in the posterior temporal C A double arm suture is placed into the tendon (right eye,
quadrant. surgeons view).
B The anterior (or if you choose the entire) tendon is D The tendon is secured to sclera
engaged on a hook (right eye, surgeons view).
231
Chapter 9
232
Surgery of the obliques
233
Chapter 9
B
Figure 19
Exposure of the superior oblique from the trochlear cuff to
the insertion after a cuffed limbal incision and tagging and
disinsertion of the superior rectus.
234
Surgery of the obliques
235
Chapter 9
A B
C D
E F
Figure 21
A Incision for exposure of the superior oblique tendon at E The tucker is adjusted pulling the loop of the tendon up
the insertion. until the intended amount of tuck is achieved and a
B Exposing the superior oblique insertion. suture secure the tuck at the borders of the tendon. The
C The superior oblique insertion is engaged on a hook. intended amount of insertion is determined after confir-
D The hook of the tucker engages the tendon. mation that the superior oblique traction test is equal or
slightly tighter on the operated side.
F A second suture secures the tuck and the tip of the loop
is sutured to sclera.
236
Surgery of the obliques
G H
I
Figure 21, contd
G conjunctiva is closed.
H A free hand tuck can be done after pulling up the lax ten-
don. A 5-0 merseline suture joins the arms of the loop
near the base.
I When performing a tuck with either technique, a loop
should be left in the initial knot so that it can be undone
easily in the event that the tuck must be adjusted
because it is too loose or too tight. When the tuck is the
correct amount, the knot is tied and a second suture may
be added.
237
Chapter 9
A B
C D
E F
Figure 22
A The superior oblique tendon. D The distal tendon is excised.
B After the loose tendon is elevated, a double arm suture E The tendon is tied securely to the insertion, after adjust-
is placed at the intended amount of resection. ment, if needed, based on superior oblique traction test-
C The tendon is cut distal to the suture and the suture is ing.
passed through the tendon insertion. F The excised tendon.
continued.
238
Surgery of the obliques
G H
239
10
Marginal myotomy:
technique and indications
Historical review
Before the advent of uniform, strong, fine-
gauge sutures with sharp swaged-on needles, partial
or incomplete myotomy was a commonly employed
technique for weakening an extraocular muscle. This
technique has now been superseded by measured
recession, which is the method of choice now for
weakening the rectus muscles in all but a few specif-
ic instances. It is important, however, for the strabis-
mus surgeon to understand the principle of marginal
myotomy and to be familiar with this technique for
use in special cases.
Six types of myotomy that have been
employed for reducing the effect of a rectus muscle
are shown in Figure 1. Three of the procedures (1, 3,
and 5) fail to cut all of the fibers of the muscle and
therefore would not be expected to lengthen the mus-
cle. In contrast, techniques 2, 4, and 6 interrupt all of
the muscle fibers at some point and would be expect-
ed to lengthen the muscle.
Figure 1
1 Central myotomy
2 OConnor triple cut myotomy
3 Incomplete marginal myotomy
4 Overlapping marginal myotomy
5 Multiple incomplete marginal myotomies
6 L-shaped overlapping double marginal myotomy
241
Chapter 10
Figure 2
The preparation for recording the muscle lengthening effect
of a marginal myotomy.
242
Marginal myotomy
A B
C D
Figure 4
A Double 80% overlapping marginal myotomies C A central 80% tenotomy produced lengthening of
seperated by a distance equal to 30% of the 0.06 times the muscles width.
muscles width produced lengthening of 0.9 D Two incomplete marginal myotomies, each
times the muscles preoperative width. including 40% of the muscles width combined
B Multiple, nonoverlapping marginal myotomies with an 80% central tenotomy displaced from the
produced lengthening of 0.03 times the muscles two previous myotomies by 30% of the muscles
width. width, produced lengthening of 0.5 times the
muscles width.
243
Chapter 10
Figure 5
A The muscle is exposed in the usual manner and two
hemostats are each placed 80% of the way across the
muscle (or tendon) from opposite borders. The hemo-
stats are plced 3 or 4 mm apart.
B The posterior hemostat is removed, and scissors are
used to cut across the muscle in the crushed area. By
cutting the muscle in the crushed area, bleeding is kept
to a minimum.
C The hemostat nearer the insertion is removed, and the
B muscle is cut along the crushed area using small snips
with scissors.
D Noticeable lengthening of the muscle will occur. Any
bleeding is controlled with pressure.
E After the distal myotomy has been performed, in a very
tight muscle, a No. 15 Bard Parker blade can be used to
divide the tendon fibers, cutting against the muscle hook.
This can be accomplished with a scraping motion with
the knife blade at nearly right angles to avoid scleral per-
foration.
244
Marginal myotomy
Figure 6
A Marginal myotomy performed on an already recessed lateral rectus muscle provides muscle
lengthening without sacrificing the arc of contact.
245
Chapter 10
Figure 6, contd
B Left hypotropia has occurred after placement of an exoplant and encircling band near the
inferior rectus insertion of the left eye. It has been treated with a double 80% marginal
myotomy of the left inferior rectus.
Other considerations
The concept that myotomy is effective only if with vertical tropia and diplopia.* The average pre-
the muscle is effectively lengthened is being chal- operative deviation was 8 prism diopters. The aver-
lenged. Hertle and associates have demonstrated a age correction at six weeks was 5 prism diopters.
damping effect on nystagmus after detaching and Diplopia was relieved in 70% of patients.
reattaching the four rectus muscles. I continue to believe that for myotomy to be
Alan Scott described graded rectus muscle effective for larger angles and for the longer term,
tenotomy (disinsertion) for treatment of small angle lengthening of the muscle is required. But these
vertical strabismus. He made successive small cuts observations of expert strabismologists must be taken
until the desired results were achieved. Biglan per- seriously when considering the effectiveness of par-
formed a 60% disinsertion of the superior rectus mus- tial myotomy for relieving symptoms of small angle
cle, mostly from the temporal border, in 24 patients strabismus.
* Yim Bin Hye, Biglan A, Cronin TH. Graded partial tenotomy of the vertical rectus muscles for treatment of hypertropia. Trans Amer
Ophthalmol Soc, Vol 102, 2004, pp. 169-176.
246
11
Faden operation
(posterior fixation suture)
247
Chapter 11
A B
C
D
Figure 1
A The eye is shown from above the medial rectus on top. D As the eye moves back to the primary position, the medi-
B With the eye in abduction, the medial rectus is stretched. al rectus behind the suture is loose or redundant. This
C The posterior fixation suture is placed with the medial would theoretically weaken the effect of the medial rectus
rectus on the stretch. in the primary and thereby reduce the esodeviation.
visual activity thereby reducing the likelihood of less important than down gaze including the reading
diplopia (Figure 2). position (except in certain exceptions) this procedure
Although not treating yoke muscles, a PFS is less likely to be indicated for the superior rectus
may be placed on the normally acting inferior rectus (Figure 3).
in case of weakness of the other inferior rectus. In I have used a posterior fixation on the four hor-
this example, there is no secondary deviation effect, izontal rectus muscles as a means of treating nystag-
only the pure limitation of movement or mus. The few cases that I treated were not success-
pseudoparalysis of the sound muscle treated with ful. Recession of the four horizontal rectus muscles
the PFS. This would result in the two eyes being has been effective for treatment of nystagmus, and
more nearly matched in down gaze thereby avoiding Hertle has reported success with simple disinsertion
or at least reducing diplopia. Because far up gaze is and reinsertion of the muscles.
248
Faden operation (posterior fixation suture)
Figure 2
A The muscle is secured to underlying sclera at or just C The new effective insertion creates a reduced lever arm
behind the equator. for the muscle. This reduced lever arm results in the
B The extent of rotation of the eyeball is restricted by the need for increased innervation for the muscle to achieve
suture compared to movement without the suture in its full (though reduced) rotation.
place.
249
Chapter 11
OD OS
OD OS
OD OS
OD OS
Figure 3
A The eyes are aligned in the primary position. D With a posterior fixation suture placed on the normal left
B The right eye has deficient depression from a right inferior rectus, the movement downward in this eye is
inferior rectus paresis. somewhat limited making it more nearly match the right
C In far down gaze, the left eye moves normally, resulting eye. The yoke of the left inferior rectus, the right
in an increasing right hypertropia and diplopia in the superior oblique, a depressor of the right eye, would also
reading position. receive more innervation to down gaze.
250
Faden operation (posterior fixation suture)
B
A
Figure 4
A The rectus muscle may be tagged with sutures at the
insertion and detached. Two sutures are then placed in
sclera at or just behind the equator to secure the muscle
Figure 5
at the borders. Non-absorbable suture, 5-0 or 6-0 is A After obtaining adequate exposure, the muscle is retract-
used. ed and a 5-0 or 6-0 non-absorbable suture is placed at
B The posterior fixation suture sutures are tied securely. the muscle border at or just behind the equator. A limbal
C The muscle is reattached at the insertion. incision is a good choice to gain best exposure. The flap
is being held with forceps. Better exposure is obtained
with a Barbie retractor.
B After placement of the sutures, the muscle looks relative-
ly undisturbed. (An optional single central suture is
shown)
251
Chapter 11
A Figure 7
Recession has been carried out and then the posterior fixa-
tion suture is placed. This means that the length of muscle
between the PFS and the muscle origin is longer, thereby
achieving both the recession effect and the posterior fixa-
tion suture effect.
Adjustable faden
Alan Scott has suggested a novel method for creating
a posterior fixation suture in a way that is potentially
adjustable. To do this, a double arm suture is placed
in the muscle at about the point where a posterior fix-
ation suture would be placed. The muscle distal to
this suture is then excised! This means that the mus-
cle is not shorter, it just inserts closer to the origin as
B with the posterior fixation suture. The sutures are
then brought through the muscles original insertion
and a suitable temporary knot is placed. The theory
behind this technique is that the muscle distal to the
suture placement of the posterior fixation suture is
probably irrelevant anyway (Figure 8).
Figure 6
A For a reinforced posterior fixation suture, the belly of
the muscle is elevated and two or three suture passes
go through sclera and muscle.
B After the identical suture placement is carried out on the
other border of the muscle, the sutures are tied securely.
252
Faden operation (posterior fixation suture)
A
B
Figure 8
A A double arm suture is placed 12 to 14 mm from the C Suture ends are brought through the original insertion.
insertion, or at the point on the muscle where the posteri- D A knot that can be released for adjustment and tied
or fixation suture would be placed. securely later is placed.
B The muscle/tendon distal to the suture is excised.
253
12
Adjustable sutures:
techniques for restrictions
Overview
Adjustable sutures were used commonly in the muscle securely to sclera at a predetermined point
early years of strabismus surgery. They were used out based on patient history, work-up, and intra-operative
of necessity rather than choice. Sutures were coarse findings.
and needles were heavy with the result that muscles I use adjustable sutures in fewer than 10% of
could not be attached to sclera either accurately or cases. This is actually an increase from about 3%
safely. Sutures were anchored in conjunctiva and earlier. This increase is due to the use of the tandem
Tenons with the muscle lying on but not attached to adjustable that actually requires adjustment only 25%
sclera. Adjustments may not have been precise in of the time. When adjustable sutures are used they are
these patients, but were necessary to obtain the best limited to older children and adults with conditions
possible results. including: previous unsuccessful surgery, thyroid
When catgut sutures with swaged-on needles myopathy, diplopia after successful cataract surgery,
became the standard for strabismus surgery, strabismus after trauma, restrictive strabismus, etc.
adjustable sutures were no longer employed. These For treatment with an adjustable suture, a muscle
sutures had the advantage of being absorbable and the should have contractile power to rotate the eye and
needles were finer making for more accurate surgery. should be working against an antagonist muscle
The disadvantage of catgut sutures was that they capable of relaxing.
broke easily making them unsuitable for the
adjustable technique. Actually a suture broke once in Technique for the adjustable
almost every case during the course of surgery, suture
especially when an assistant was allowed to tie a knot.
There are several effective techniques for
During the 1960s when I started doing strabismus
placement of an adjustable suture. Regardless of the
surgery adjustable sutures were neither done widely
technique used, the following principles remain
nor talked about.
constant.
Adjustable sutures were re-discovered and
1. The muscle is secured with suture that is
popularized by Jampolsky after synthetic absorbable
sufficiently strong to withstand postoperative
sutures became available in the 1970s. This
manipulation.
technique is currently used widely for treatment of a
2. The muscle is attached to the globe usually at
wide variety of strabismus conditions. Their use,
the muscles insertion stump in a hang loose
however, is not universal. Some surgeons use
manner.
adjustable sutures for nearly every case stating that
3. The suture anchoring the muscle is secured in
better results are achieved if a second chance is
a way that it can be easily loosened and then
available or saying that better results can be achieved
re-tied at the time of adjustment.
if the postoperative alignment can be determined with
4. The suture is able to slide through the site of
the patient awake and cooperating. Other surgeons
attachment to the globe allowing the muscle
use them either sparingly or not at all. The belief
to slide back or be pulled forward.
among these surgeons is that in most (all) cases more
precise surgery can be accomplished by attaching the
255
Chapter 12
A B
C D
E
F
Figure 1
A The muscle is exposed. E The sutures are brought through the muscles stump.
B The suture is placed 1 to 1.5 mm from the insertion (a F If a bolster is used, the suture is brought through after
handle-suture as shown in Figure 2 is placed in all putting the needles through conjunctiva to produce a
cases). conjunctival recession (as shown) or through conjunctiva
C The suture is secured with a central bite which is tied overlying the stump.
and locking loops are placed at the borders. G The suture is tied over the bolster (if used) or on con-
D If the muscle is tight, it is cut from the globe with a junctiva.
scalpel, cutting against a muscle; otherwise scissors are
used.
256
Adjustable sutures: techniques for restrictions
5. The incision in conjunctiva should be made only suggest leaving the eye in the same alignment
so that the surgeon can access the suture at the that is preferred when standard surgery is performed;
time of adjustment and then close the that is, a slight overcorrection.
conjunctiva satisfactorily with the patient A handle of 6-0 Vicryl placed in sclera at the
awake. limbus as a means of grasping and manipulating the
6. The use of adjustable sutures are effectively globe during adjustment is shown in Figure 2. The
limited to the rectus muscles (Figure 1). handle must be exposed at the conclusion of surgery
In every case of adjustable suture, a so-called regardless of which type of incision has been used. A
handle suture is placed in superficial sclera usually forceps grasping the handle allows relatively easy
near the limbus. This is used for stabilizing the eye, rotation and stabilization of the globe during surgery
securing the muscle, and in rotating it at the time of and during adjustment. A three-cornered limbal
adjustment. The suture can be temporarily tied with incision may be used that can be taken down at the
a bow know, a slip knot, or a noose like cinch knot time of adjustment and repaired when the adjustment
that can be secured tightly or loosened to slide up and is completed. A sliding knot over the sutures
down the suture as needed during adjustment. suspending the muscle can be helpful during
Use of an adjustable suture begs the question, adjustment. The sliding knot is secured at surgery
Where should the eyes be placed at the time of when the muscle is at the intended position and the
adjustment?. While there is no reliable answer to suture ends are tied. At adjustment, the knot may be
this question, I tend to leave the eyes in the alignment loosened and slid toward the cornea while the patient
I would like to achieve at the same period looks in the opposite direction as the globe is
postoperatively if the muscle had been firmly stabilized with the handle suture if the recession
attached to sclera at the time of operation. Patients effect is to be increased. If the recession effect is to
with postoperative diplopia are adjusted to a diplopia- be lessened, the suture holding the muscle is pulled
free position. Non-fusing exotropic patients are left up and the slip knot is slid toward the muscle. When
straight or slightly exotropic; non-fusing esotropic a cul-de-sac incision is used, a handle suture is placed
patients are left straight or slightly esotropic. at the upper insertion in case of an inferiorly placed
Because I never use an adjustable suture in a patient incision. For adjustment, this suture is pulled up to
treated surgically for intermittent esotropia, I can center the incision over the muscle stump.
A B
Figure 2
A The handle suture. B The suture ends must be retrievable.
continued.
257
Chapter 12
C D
E F
G H
Figure 2, contd
C The handle suture stabilizes the globe during adjustment. G The muscle is advanced or it drops back sliding through
D A three cornered limbal incision may be used. the slip knot which is tightened when the muscle is in the
E The sutures may be secured with a slip knot. intended position.
F The amount of hang back can be measured. H Both the handle suture and the adjustable suture are led
out through the cul-de-sac (inferior) incision shown.
I The handle suture lifts the incision over the muscle inser-
tion.
258
Adjustable sutures: techniques for restrictions
A B
Figure 3
A Two 6-0 vicryl sutures are placed 1.5 and 2.5 mm from C The incision is closed with the adjustable suture ends
disinsertion. exposed. If no adjustment is needed the adjustable
B The muscle is detached and the proximal (nearer the sutures are cut.
insertion) sutures pass through the stump producing a continued.
hang loose recession, placing the muscle where the
surgeon thinks best. The distal sutures are brought
through the stump outside the first sutures - they are left
untied.
259
Chapter 12
Figure 3, contd
D If at adjustment the muscle is to be let back the first E If the muscle is to be adjusted closer to the insertion, it is
suture is cut and the muscle is adjusted using the sec- simply pulled up with the adjustable suture. The first
ond suture. suture is redundant.
Adjustable suture
considerations
Important considerations for the adjustable the eye appropriately and then carrying out cover test-
suture technique are ease of adjustment, accuracy of ing. If the patient is able to respond, I ask whether the
final alignment, and patient comfort during and after patient sees two and by careful adjustment work to
surgery and during adjustment. Enthusiasm for use of eliminate the diplopia. If general anesthesia is used,
adjustable recession and resection varies widely I merely attempt to center the eye and carry out the
among even the most experienced surgeons. Some adjustment later. The suture may be adjusted on the
never use this type of surgery stating that the tech- day of surgery, or in the recovery room an hour or
nique is absolutely unnecessary and that excellent more after surgery if the alignment is significantly
results can be obtained without using adjustable different than intended. The most common and most
sutures. Other equally experienced and competent productive time for adjustment is approximately 24
surgeons use adjustable sutures in every patient who hours after surgery, either at the bedside for those
will cooperate for adjustment with few exceptions. patients who are admitted to the hospital or in the
The majority of surgeons probably occupy the middle clinic for outpatients. Topical anesthesia with
ground, using the adjustable suture technique only in proparacaine hydrochloride, tetracaine, or 5%
selected cases. I perform adjustable suture surgery on Xylocaine may be used. A lid speculum is helpful to
less than 10% of adult patients undergoing strabismus give exposure while picking up the suture ends.
surgery. Tying forceps are used to grasp the suture holding the
As with the use of adjustable sutures, timing of muscle and a fine utility forceps is used to grasp the
adjustment varies widely among surgeons. When a handle suture. Scissors are used to trim the suture
patient has topical or perilimbal anesthesia with min- ends after the adjustment has been completed. One
imum sedation, I prefer to carry out the adjustment surgeon I know claims to be able to adjust a muscle as
while the patient is on the operating table, positioning late as 10 days after surgery. Although I believe her,
260
Adjustable sutures: techniques for restrictions
I would not attempt an adjustment that long after sur- When reoperating a patient who has undergone a
gery. From experience with a few cases of early reop- previous conjunctival recession, it is necessary to
eration, I believe that a fairly firm myoscleral union is enter sub-anterior Tenon's space at the point where
formed in just a few days. Rather vigorous manipu- conjunctiva had been recessed. The sclera in the area
lation of the globe is required to accomplish late mus- of conjunctival recession becomes re-epithelialized
cle adjustment. As with surgery performed with top- with a thin layer that adheres tightly to underlying
ical or local anesthesia, the pain during adjustment sclera. It should not be disturbed. Patients are usual-
comes mainly from traction on the muscle and is deep ly comfortable after conjunctival recession.
in the orbit. This can cause syncope and nausea. For Ointment is used twice a day after surgery (switching
adjustment it is a good idea to either have the patient to drops in the morning if ointment causes blur) and
lying down or have a bed available nearby. The no patching is necessary. It is also important to
patient may take a mild oral analgesic a few hours remember that conjunctiva becomes extremely thin
before the adjustment to make them more comfort- and friable in older patients. Even some patients in
able. their 20's may have very thin conjunctiva. Nearly all
patients 30 years and older have very thin conjuncti-
Conjunctival recession va. Therefore, it is impractical to attempt a cul-de-sac
incision in an older patient unless the surgeon has
When the conjunctiva is recessed because of
inspected the conjunctiva and has determined that it
tightness that restricts free movement of the eye, it is
could withstand the necessary manipulation.
usually necessary to move the limbal margin of the
Eyes with longstanding esotropia usually have a
conjunctiva back 5 to 7 mm or to the insertion site of
foreshortened conjunctiva that restricts abduction. A
the recessed muscle. In the rare case where conjunc-
limbal incision is made in the usual manner encom-
tival recession is performed without recessing the rec-
passing approximately 2 to 3 clock hours centered
tus muscle, the limbal margin is moved to a point just
over the muscle's insertion with radial relaxing inci-
covering the insertion of the muscle. When the con-
sion approximately 10 mm long. For closure with
junctiva is severely scarred it may be excised and the
recession, conjunctiva-Tenon's is sutured to sclera
cut edge of conjunctiva attached to underlying sclera.
with three interrupted 8-0 Vicryl sutures used. With
This step may be carried out medially as far as the
the conjunctiva sutured in place, the bare sclera is left
plica semilunaris. Because the medial conjunctiva
to re-epithelialize in a day or so.
containing the plica semilunaris and caruncle has
With a severely scarred conjunctiva, the entire
more tissue and because the medial recti are the most
conjunctival flap may be excised and the cut end of
frequently operated extraocular muscles, medial con-
conjunctiva sutured to underlying sclera and the
junctiva is the area most frequently in need of revi-
relaxing incision sites sutured to adjacent conjuncti-
sion. In contrast to reddened scars of the conjunctiva,
va-Tenon's. When medial scarring is extensive, the
clear subconjunctival cysts which occur occasionally
medial conjunctiva can be excised as far medially as
after eye muscle surgery can be removed, sometimes
the plica semilunaris. The plica is then sutured direct-
intact, without the need to recess the conjunctiva pro-
ly to underlying sclera far medially (Figure 4).
vided the overlying conjunctiva remains elastic.
A B
Figure 4
A Tight, scarred conjunctiva B For conjunctival recession A is attached to A1 and C to
C1. A third suture is placed in the center.
continued.
261
Chapter 12
C D
F G
H I
262
Adjustable sutures: techniques for restrictions
Traction sutures
When the surgeon is concerned that postopera- taken at the 6 o'clock limbus position and the 4-0 silk
tive adhesions may cause the globe to remain fixed in sutures are taken out through the upper tarsus and tied
an undesirable position, traction suture placement over a rubber or silicone peg. To fix the eye is deor-
may be used. The eye should always be placed in a sumduction, the two bites are taken at the 12 o'clock
position opposite the undesirable fixation. A chroni- position with 4-0 silk sutures, and the sutures are
cally esodeviated eye with restricted abduction brought out through the lower tarsus and tied over a
should be fixed in abduction, a Brown syndrome rubber or silicone peg.
should be fixed in adduction and sursumduction, and Some surgeons prefer to anchor traction
so on. sutures through the tendinous insertion of the rectus
In Figure 5, the right eye is to be placed in muscles. The attachment to the globe is more secure
forced abduction. Two scleral bites are taken near the with this technique. The traction sutures are placed at
nasal limbus with 4-0 silk sutures. The sutures are the insertion of the superior and inferior rectus before
brought out through the upper tarsus and tied over a fixing the eye in abduction or adduction. Traction
rubber or silicone peg with the eye in several degrees sutures are placed at the insertion of the horizontal
of abduction. The sutures are removed in 5 to 7 days. recti to fix the eye in sursumduction or deorsumduc-
Because the eye is rotated, corneal contact by the tion. The right eye is fixed in abduction and the
suture is kept to a minimum. To place the eye in sutures are brought out through the temporal aspect of
forced adduction, suture placement is reversed. To the upper lid, fixing the eye in the abducted position.
fix the eye in sursumduction, two scleral bites are
A B
C D
Figure 5
A A 4-0 black silk suture is secured to sclera. D The eye anchored in depression.
B The eye anchored in adduction. E The eye anchored in elevation and adduction with two
C The eye anchored in elevation. traction sutures placed in the insertion of the superior
and inferior rectus.
263
13
Muscle transposition
procedures
Overview
When an extraocular muscle is paralyzed it has ist, the mechanical restrictions must be eliminated
lost the ability to contract. The usual strengthening before carrying out the extraocular muscle transfer.
techniques such as resection, advancement, or tuck This release of mechanical restrictions can be done
(actually shortening procedures) do not restore the with surgery or to some extent by chemodenervation
muscles potential for normal ocular rotation. A new, with Botox.
more favorable, static position of the globe may be Extraocular muscle transfer achieves a change
accomplished after a large recession-resection proce- in the mechanics of a given muscle but innervation to
dure, but movement in the field of action of the para- this muscle remains the same as preoperatively, and
lyzed muscle is not accomplished. To remedy this, the muscle continues to obey Herings law. A suc-
Hummelsheim in 1907 devised a procedure to trans- cessful extraocular muscle transfer procedure has
fer part of the action of the superior and inferior rec- most of its effect in changing alignment in the pri-
tus muscles to the field of action of the lateral rectus mary position with only a limited effect in the field of
muscle in cases of sixth nerve palsy. action of the paralyzed muscle. This movement may
This procedure has undergone numerous mod- be due to a spring load effect created by the trans-
ifications in the last century, but most retain the basic ferred muscles and activated when the antagonist
principle of the technique as introduced by relaxes according to Sherringtons law during
Hummelsheim. The principle is that action of mus- attempts to look in the field of action of the paralyzed
cles that are normally antagonists are transferred to muscle. Some patients with acquired sixth nerve
the field of action of the muscle lying between these palsy and also some with acquired vertical rectus
antagonists. For example, the superior and inferior palsy can achieve expanded areas of diplopia-free
rectus muscles are transferred to a point adjacent to vision after muscle transfer, and others just look bet-
the lateral rectus muscle in sixth nerve palsy or to the ter in the primary position while retaining large areas
medial rectus in case of medial rectus palsy. The hor- of diplopia.
izontal recti are likewise shifted adjacent to the supe-
rior rectus in superior rectus palsy and to the inferior A review of muscle
rectus when this muscle is paralyzed. transposition procedures
Muscle transposition may be indicated in any In Hummelsheim's original transplant proce-
case where paralysis of a muscle is associated with an dure, the lateral halves of the tendons of the superior
unacceptable deviation in the primary position and/or and inferior rectus muscles are attached to the tendon
bothersome diplopia. This can occur in unilateral or of the lateral rectus (Figure 1A). In O'Connor's mod-
bilateral sixth nerve palsy, double elevator palsy, infe- ification of the Hummelsheim procedure the entire
rior rectus palsy, with an irretrievable lost muscle, tendons of the superior and inferior rectus muscles
and other causes. It should be emphasized that in are sutured to the sclera adjacent to the insertion of
cases of extraocular muscle paralysis, especially the lateral rectus and a cinch is performed on the lat-
those of longstanding, mechanical restriction can be eral rectus (Figure 1B). In a further modification of
present in the antagonist. When paralysis and O'Connor's technique the nasal halves of the superior
mechanical restrictions limiting free movement coex-
265
Chapter 13
A B
C D
E F
266
Muscle transposition procedures
H I
J K
and inferior rectus tendons are passed beneath the (Figure 1H). In Jensen's technique the superior rec-
temporal halves of the insertions and attached to the tus, inferior rectus, and lateral rectus muscles are split
sclera adjacent to the lateral rectus tendon (Figure along their long axes. The lateral half of the superior
1C). In Wiener's procedure the paralyzed lateral rec- rectus is joined to the superior half of the lateral rec-
tus is transected and the proximal tendon is split and tus and the inferior half of the lateral rectus and the
joined to the adjacent superior and inferior rectus lateral half of the inferior rectus are joined in a simi-
muscles (Figure 1D). In Peter's procedure for third lar fashion with nonabsorbable sutures. The medial
nerve palsy, the trochlea is fractured and a shortened rectus may or may not be recessed. This procedure
superior oblique tendon is sutured to the sclera near performed on appropriate muscles also has been sug-
the insertion of the medial rectus (Figure 1E). In gested for double elevator palsy, medial rectus palsy,
Hildreth's procedure the entire tendons of the superi- and double depressor palsy (Figure 1I). In Uribe's
or and inferior rectus muscles are joined with nonab- technique the medial rectus is recessed, the lateral
sorbable suture (Figure 1F). rectus resected, and the entire tendon of the superior
In Schillinger's procedure the entire tendons of and inferior rectus muscles sutured to the sclera adja-
the superior and inferior rectus muscles are sutured to cent to the resected lateral rectus insertion (Figure
the sclera near the insertion of the lateral rectus 1J). In Knapp's technique for double elevator palsy
(Figure 1G). In Beren's and Girard's technique the the entire tendon of the medial and lateral rectus mus-
medial rectus is recessed, the lateral rectus resected, cle is shifted and sutured to the sclera adjacent to the
and both superior and inferior rectus muscles shifted insertion of the superior rectus. The inferior rectus
one-half width temporally, with the temporal half of may also be recessed. This full tendon transfer may
each muscle sutured to the resected lateral rectus be used for any of the rectus muscles (Figure 1K).
267
Chapter 13
Figure 2
A A limbal incision is made for 180 degrees. This proce-
dure is shown for transfer of the superior and inferior
rectus to the lateral rectus, but it may be done for any of
the rectus muscles.
B The three rectus muscles are exposed.
C For a full tendon transfer, one or two sutures are placed
1 or 2 mm behind the insertion of the muscle to be trans-
ferred using one double arm or two single arm sutures.
continued.
268
Muscle transposition procedures
D E
F G
Figure 2, contd
D After detaching the muscles, they are reattached to scle- F For a more powerful muscle transfer, the borders of the
ra concentric with the limbus with one border of the mus- muscle can be joined with a non-absorbable suture 8 mm
cle just touching the edge of the insertion of the para- behind the insertion of the paralyzed muscle, or these
lyzed muscle. transferred edges can be sutured to sclera adjacent to
E For a half muscle transfer, the muscle is split with a mus- the paralyzed muscle. This can be done with a full or a
cle hook and is separated backward for 15 mm. A suture half tendon transfer.
is placed in the half of the muscle to be transferred, the G The antagonist rectus may be recessed.
muscle is detached and reattached just touching the H Botox may be injected in the antagonist.
edge of the insertion of the paralyzed muscle. Care
should be exercised to spare the remaining ciliary artery.
269
Chapter 13
Figure 3
A large exotropia in a patient who at surgery was found to have no left medial rectus.
270
Muscle transposition procedures
A B
Figure 4
A The scleral strip is sutured 5.5 mm from the limbus, the B The scleral strip pulls the vertical recti toward the empty
vertical rectus is split, and the scleral strip is passed medial rectus insertion site. The antagonist is recessed.
through the split muscle. C The ends of the scleral strip are joined with non-
absorbable sutures.
Figure 5
Postoperative alignment is good.
271
Chapter 13
Knapp procedure
The Knapp procedure remains a popular tech- The procedure starts with a large limbal inci-
nique for muscle transposition. It is useful for treat- sion or equivalent (Figure 6). The medial and lateral
ing double elevator palsy especially when there is no rectus muscles are secured with two single arm or one
mechanical restriction to elevation. This technique double arm suture, are detached and reattached at the
employs upward shift of the medial and lateral rectus corner of the superior rectus insertion. Subtle differ-
muscles to a point adjacent to the corners of the inser- ences in the alignment of the insertions of the trans-
tion of the superior rectus muscle. It differs from ferred muscles can be employed to modify both the
other transpositions of the full tendon in that the line horizontal and the vertical pull. In theory the higher
of insertion of the transposed muscles is more or less the placement of the new insertions, the more the
parallel to the borders of the superior rectus. This is upward pull on the eye. In addition, location of the
in contrast to those full tendon transfer procedures placement of the new insertions, especially that of the
that have the new insertion of the transferred muscles lateral rectus muscle can affect the horizontal align-
concentric with the limbus. This may make a differ- ment. I produced a large transient exodeviation in
ence in the pull of the transposed muscle, especially one patient after doing this procedure for double ele-
if the concentric insertion transfer is reinforced with a vator palsy. The conjunctiva is closed in the usual
suture joining the transposed muscle to the paralyzed manner.
muscle 8 mm posterior to the insertion.
Figure 6
A A large limbal incision ( or equivalent) is made. C The incision is closed with 8-0 absorbable suture.
B After placing one or two sutures in the medial and lateral
rectus muscles, these muscles are detached and reat-
tached adjacent to the border of the superior rectus mus-
cle.
272
Muscle transposition procedures
Figure 7
A The location of the limbal incision.
B The superior oblique tendon engaged on a muscle hook.
At this time a small clamp is placed on the superior
oblique tendon and it is cut as close to the insertion as
possible.
C The tendon is sutured to sclera at the upper border of
the medial rectus muscle.
273
Chapter 13
Figure 8
A For treatment of a chronic right head tilt without oblique B For treatment of chronic left head tilt without oblique
muscle dysfunction, in the right eye, the superior rectus muscle dysfunction, in the right eye, the superior rectus
is shifted one muscle width nasally and the inferior rectus is shifted one muscle width temporally and the inferior
is shifted one muscle width temporally. In the left eye, rectus is shifted one muscle width nasally. In the left
the superior rectus is shifted one muscle width temporal- eye, the superior rectus is shifted one muscle width
ly and the inferior rectus is shifted one muscle width nasally and the inferior rectus is shifted one muscle width
nasally. temporally.
274
14
Botox (Botulinum A toxin)
Overview
Extraocular muscle action must altered in some a general neural shut down. The therapeutic para-
way in order to change alignment of the eyes when lytic effect of botulinum toxin on extraocular muscle
treating strabismus. This is done by surgery usually function is dose related with minuscule doses produc-
in the form of recession, resection, transfer, myotomy ing maximum effect in 5 to 7 days after injection of
and tenotomy,. Ocular alignment may also be influ- the drug. Although 6 to 9 months may be required to
enced by optical correction with plus lenses to reduce recover completely from the effects of the toxin, the
an esodeviation in refractive esotropia and high AC/A useful effect may be much shorter for a variety of rea-
and also minus lenses in excess of the patients refrac- sons including toxin integrity, administration tech-
tive error to reduce an exodeviation. Cholinesterase nique, and severity of disease. The effect of the toxin
inhibitors used topically can increase the efficiency of can be reduced by the early injection (within thirty
accommodation and thereby reduce accommodative minutes) of antitoxin. However, I do not know of
convergence and the associated esodeviation. clinical facilities having this drug on hand. Repeated
Another way of weakening the effect of an injections of botulinum toxin tend not to be recog-
extraocular muscle is the injection of a selected drug nized by the human immune system, but antibodies
into the muscle itself. This had been tried in the past have been found in some patients. I have personally
using agents such as alcohol or local anesthetics injected more than 1,000 cumulative units of Botox
which were either too successful causing irre- into several blepharospasm patients over the course
versible muscle paralysis or transient. Then in 1972 of several years with no apparent adverse side effect.
Alan Scott began injecting extraocular muscles in the The local effect of the toxin can be prevented by prior
laboratory in search of a clinically effective agent. toxoid immunization.
He tried several including Cobra toxin, finally settling The large botulinum molecule is fragile and is
on botulinum toxin (type A). Human studies began in susceptible to damage from shaking and frothing;
1977. The drug was first released for investigational therefore, it should be reconstituted, drawn up, and
use and was finally placed on the market in 1989 for injected gently. Botox is supplied in vials that contain
the treatment of blepharospasm and strabismus in 100 units of freeze-dried toxin. Each unit contains
patients over 12 years. The sales and distribution of about 0.25 ng (billionths of a gram) of toxin. The
botulinum A toxin was taken over later by Allergan toxin is stored in a freezer until used.
Pharmaceutical and is now marketed for a wide vari- The freeze-dried drug is reconstituted with
ety of uses under the name Botox. non-preserved normal saline. Four ml of 0.9% NaCl
are injected into the bottle with the toxin. When
The drug reconstituted according to specific dilution instruc-
Clostridium botulinum is a large, aerobic, gram tions present on each vial, the concentration is 25
positive, rod shaped organism. Of the eight immuno- U/ml (or 2.5 U/0.1ml). The reconstituted Botox
logically distinguishable exotoxins three types (A,B, should be used within a few hours of mixing to retain
and E) are commonly associated with human toxicity. its maximum therapeutic effect. The human LD/50
Paralysis of a muscle by botulinum is caused by the for Botox is approximately the full contents of 20
inhibition of the release of acetylcholine (Ach). In vials, making this drug potentially less dangerous
the case of accidental intoxication, death is caused by than aspirin!
275
Chapter 14
276
Botox (botulinum A-toxin)
277
Chapter 14
A B C 4 CC
NaCl 0.9%
Off On
1 CC
diluted Botox
Figure 1
A The vial of Botox contains 100 units of freeze dried toxin. D The appropriate amount of toxin is drawn up into a tuber-
B NaCl 0.9% without preservative is used for dilution. culin syringe with another 27-gauge needle which is then
C Four ml of the NaCL 0.9% is drawn up in a small syringe exchanged for a 2 inch Teflon-guarded 27-gauge needle.
using a 27-gauge disposable needle and is then injected E The needle hub is connected to a lead from the EMG
slowly into the bottle of freeze dried toxin. The bottle is recorder and the second lead is attached to the patients
not shaken and the liquid is not frothed. forehead. (If injection is done for blepharospasms there
is no need for EMG control and a regular 27-gauge dis-
posable needle may be used).
278
Botox (botulinum A-toxin)
B C
Figure 2
A With the eye stabilized with fine-toothed forceps and the B The patient continues to look away from the muscle to
patient looking away from the muscle to be injected. The be injected while the needle is advanced about one inch.
Teflon-guarded needle is thrust through conjunctiva just C With the needle advanced to this point the patient is
behind the level of the muscle's insertion. asked to move the eye slowly in the direction of the mus-
cle to be injected while the surgeon listens for the crack-
ling indicating neural activity.
continued.
279
Chapter 14
D E
Figure 2, contd
D The patient is then asked to look slowly away from the F A patient with right sixth nerve palsy would look like this
muscle to be injected while the surgeon advances the before injection of the right medial rectus.
needle tip carefully into the muscle and injects the toxin.
E Immediately after this, the needle is removed.
280
Botox (botulinum A-toxin)
away from the muscle being injected. After the nee- Botox for the treatment of
dle has entered the sub-conjunctival space the EMG
recorder is turned on. The needle is advanced slowly blepharospasm
and steadily with the bevel facing away from the scle- The most common use of Botox in our clinic
ral surface. The surgeon during this maneuver listens has been for treatment of benign essential ble-
carefully for the crackling sound indicating nerve pharospasm and other annoying, even disabling facial
activity. When the needle has been advanced approx- spasms and ticks. Between 80 and 100 patients seen
imately one inch or when the surgeon first hears the on a regular basis receive a total of more than 300
crackling sound, the patient is asked to look slowly Botox injections each year. Satisfactory results are
in the field of action of the muscle being injected. A obtained in nearly every case allowing the patient to
sharp increase in the audible electrical activity indi- engage in normal life activities without the embar-
cates that the bare metal tip of the needle is adjacent rassing spastic movement of their facial muscles. The
to the muscle's motor end plate. It takes a practiced beneficial effect of Botox injection lasts on average 3
ear to differentiate noise from the muscle's neural to 6 months. Patients have received repeat injection
activity. At this point, the patient can be asked to look up to 30 or more times without apparent adverse
away from the muscle to be injected. A very short effect.
advance of the needle will result in the needle enter- Because blepharospasm and facial myokymia
ing muscle substance at which time the toxin is inject- can be associated with brain stem disease and multi-
ed. This can mean an injection from as little as 1 unit ple sclerosis among other serious neurologic disease,
to a dose of as much as 5 units. I have not exceeded we require that patients be under the supervision of
this upper limit. The level of neural activity crack- their family physician or a neurologist before provid-
ling diminishes dramatically upon injection. I am ing Botox treatment for blepharospasm.
not sure whether this is from the mechanics of injec- The surgical treatment of blepharospasm is a
tion or from the cessation of neural activity. Since the formidable undertaking with potential complications
muscle continues to act normally for hours to days including facial paralysis and corneal exposure. With
after injection I suspect it is the former. The patient the advent of Botox, the indications for surgical treat-
ordinarily has no discomfort after injection, but a ment of blepharospasm are less.
small subconjunctival hemorrhage may occur.
Before injecting a strabismus patient with Injection techniques for
Botox, the patient (or parents) should understand that blepharospasm and facial
the benefits of this treatment depend on an early over- spasm
correction that could be alarming if forewarning had Benign essential blepharospasm is treated with
not been provided. Patients should also be warned five 2.5 unit doses of Botox injected subcutaneously
that unintended toxin spread could cause other stra- at the medial and lateral aspect of each lid and at the
bismus and even ptosis on a temporary basis. Also, lateral canthus (Figure 3). Care should be exercised
the beneficial results of Botox treatment for strabis- to avoid injecting the mid portion of the upper lid so
mus may be only temporary. A woman I treated suc- as to avoid paralyzing the levator palpebri and caus-
cessfully with Botox for small angle strabismus, went ing ptosis. In cases where the spasm spreads to other
to the bus station and in a photo kiosk took pictures of facial muscles and even to the neck, similar 2.5 unit
her alignment on a weekly basis recording the return injections are given subcutaneously at the site of the
of the original angle of strabismus over a period of a spasm. I have give up to 50 units at one treatment.
few months and sending them to me! Injection should not be made inferior to the nasolabi-
al fold! Injections here cause lip droop that in turns
leads to very annoying lip biting by the patient.
Figure 3
A The site of subcutaneous injection for blepharospasm.
continued.
281
Chapter 14
B C
Figure 3, contd
B A typical appearance before injection. D An example of sites around the face that would be inject-
C A typical appearance a few days after injection. ed in a typical case of hemifacial spasm.
282
Section 4
285
Chapter 15
image is the largest picture that can be imaged on a mechanism for follow up. At a third visit two weeks
computer screen. For telemedicine carried out on the after this, the local doctors were given a simple com-
internet there is no need to have a denser image. The puter, and a digital camera. The doctors were
issue is complicated by the fact that most digital cam- instructed to obtain digital images of patients in the
eras are considered better or more valuable if they are nine diagnostic positions, plus head tilt to the right
able to take pictures with higher pixel numbers. For and left, and other pictures showing head posture, etc.
example, we see cameras with 3.0, 4.0, or 5.0 or more They were instructed to send these pictures via email
megapixel capacity. Of course, these pictures will be along with a brief clinical history. At the consultants
of higher quality and will provide clearer printed pic- (my) end, these pictures were arranged in an album
tures, particularly if enlarged but they offer absolute- and then printed out as shown in the Figure 1. A diag-
ly nothing in terms of a better picture for internet- nosis, suggestions for further evaluation when indi-
based telemedicine conducted on a computer screen. cated and example of a treatment plan were sent back
Actually, these pictures with high number of pixels via email for each of these patients, but the doctors
are a great detriment to telemedicine because they were advised to withhold any specific treatment
demand so much space that transmission time is based on my diagnoses and opinions.
slowed and storage space is used up. Some large At a fourth visit, 15 patients who had received
images are even blocked. For strabismus manage- consultation via telemedicine were examined and a
ment it is important to place the digital camera on the diagnosis and treatment plan for each was arrived at
lowest setting which is 640 x 480 or sometimes listed by me. These were then compared with the telemedi-
as TV, or PC. These pictures have even been used cine diagnoses and treatment plans and it turned out
for textbook illustrations (throughout this book for that the agreement was nearly perfect.
example, especially chapter. 15) and are perfectly As a means of further confirmation, ten patient
adequate as long as enlargements are not too great. consultation requests with the history and complete
These low pixel pictures have the ability to cap- clinical pictures were sent to a panel of ten strabismus
ture close-up pictures that allow enough detail for experts. They were asked to make a clinical diagno-
external and in some cases, even anterior segment sis and suggest a treatment plan. The clinical diag-
evaluation. We have evaluated, for example, worms noses agreed in more than 90% of cases and treatment
in the anterior chamber, uveitis, cataract, corneal dys- plans were virtually identical in 50% of the patients
trophies, and more. and were similar and logical in the others. Based on
this information, the doctors in Cuba were advised to
Computer continue to send patients and to work with the
The computer for telemedicine can have a telemedicine program regarding diagnosis and treat-
processor of moderate speed and a RAM of between ment.
128 and 512 MB. An adequate RAM is 256 MB. For New programs using email and digital images
the hard drive, a capacity of 20-80 gigabyte is ideal, were then established in Romania, India, Albania, and
but lower storage capacity computers are certainly the Dominican Republic. By the fall of 2002, approx-
useable since the process depends more on transmis- imately 2,000 communications had taken place with
sion than storage. Internet connectivity works better, the telemedicine partners. At this time, the program
of course, with broadband connection with 100 kilo- was given to ORBIS International. It was adopted as
bytes per second, but I have used dial-up connections a formal ORBIS program called Cyber-Sight. In
at 19.2 kilobytes per second successfully. It is much order to facilitate the transfer of patient information,
more important to use small file pictures than it is to a server-based patient submission format was estab-
have an ultra fast connection. lished (see Figure 2). Beginning in the spring of
2003, consultations were submitted using this format.
Start of Cyber-Sight Between the spring of 2003 and the summer of 2005,
nearly 1,700 patients and 4,000 communications were
How did I personally start with telemedicine? I carried out using this new technique.
visited Havana, Cuba in 1998 with an ORBIS hospi- The format for patient presentation is shown in
tal based program. While there it occurred to me that Figure 3. This includes a greeting to the person sub-
even with a successful 5 day visit, maintaining con- mitting the consult and space for the patients name,
nection with the doctors would be difficult. At a first gender, birth date, visual acuity, and refraction. The
visit, I was able to determine specific equipment partner requesting the consultation also has the
needs. This prompted a second visit approximately opportunity to select from a pull-down box the sub-
two weeks later. With additional equipment, it was specialty most appropriate for the patient. In addition
possible to complete the surgery schedule. However, to strabismus, consultation is offered in glaucoma,
even this was unsatisfying in that there still was no retina, cataract, neuro-ophthalmology, uveitis,
286
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Figure 2 The formatted page used for initiating a consultation with Cyber-Sight
287
Chapter 15
retinoblastoma, ophthalmic genetics, oculoplastics, left eye. The final four pictures are for other views
cornea, and more. In this program, the majority of the that could include head posture or other significant
patients, especially at the beginning, were in the cat- characteristics of the strabismus. After these pictures
egory of pediatric ophthalmology and strabismus. are uploaded three at a time, the submitting partner
A space below this section is reserved for a provides a diagnosis and a tentative treatment plan
patient history. This includes chief complaint, prior and optional further comments (Figure 3). The case
medical history, a narrative of the measurements and is then submitted. At this time the consulting mentor
evaluation, family history, prior surgery, etc. The is alerted by email. The mentor then sees a screen
next repository for information includes a series of which provides a complete patient presentation
cartoons which allow for the uploading of 17 images. including narrative and appropriate pictures (see
The first nine images are of the diagnostic positions. Figure 4). The mentor provides an answer, starting a
Below that are images of the head tilt, 45 right and dialogue that could include several additional com-
left, and notation of the patient fixing with a translu- munications. The case is closed eventually by the
cent occluder placed over the right eye and then the partner.
Figure 3
For the submission of a strabismus patient, Cyber-Sight partners are prompted to upload up to 17 images. These are low
density 640 x 480 pixel images.
288
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Figure 4
The array of patient pictures
289
Chapter 15
100%
Table 1
290
Telemedicine: distance medicine
Measurements
20 pd ET - up
40 pd ET - primary
50 pd ET - down
ECA0022
History Comment
3 year old boy This boy has a V pattern congenital esotropia
fixes and follows well with both eyes with bilateral overaction of the inferior obliques
In each case, refraction refraction OD +1.50 + 1.50 x 90 OS +1,50
+1.50 x 90
and underaction of the superior obliques, Also
note the antimongoloid fissures. This feature
was done after could be associated with pulley heterotopy. In
The eye examination is normal except for signif- this case the medial pulleys would be displaced
cycloplegia unless stated icant fundus extorsion and the V esotropia with upward and the lateral pulleys displaced down-
otherwise 4+ overaction of the inferior obliques and signif- ward. According to the pulley theory the V is
caused by the displacement of the rectus muscle
icant underaction of the superior obliques.
action. The V in this case would be treated
with a medial rectus recession with down shift of
the reinserted muscle. Pulley displacement must
be confirmed by coronal imaging of the mid-
orbit. The traditional treatment for a case like
this is bimedial rectus recession with bilateral
inferior oblique weakening. However, some
cases so treated have persistent overaction of the
inferior obliques. This could be caused by pul-
ley displacement. Combined inferior oblique
weakening and down shift of the recessed medial
recti could also be done. As an extreme, weak-
ening of the inferior obliques and tuck of the
superior oblique could be done, but I think that is
too much surgery.
291
291
Chapter 15
292
Telemedicine: distance medicine
Measurements
Orthotropic by light reflex
GUM0060
History Comment
This six month old girl was presented by her par- In a case like this it is difficult to do a cover test
ents because they were concerned about the eyes to confirm the alignment. In place of a cover test it is
turning in. She is otherwise healthy and there is no important to observe the corneal light reflex. If the
family history of strabismus. The eye examination is light reflex is in the center of the pupil it is likely that
normal and cycloplegic refraction is OD +1.00 D and the eyes are aligned in spite of the apparent esotropia
OS 1.00 D. that is caused by the wide nasal skin folds obliterating
view of the nasal conjunctiva. Pulling the skin over
the bridge of the nose forward will have an instant
straightening effect, but it usually causes an infant
to become fussy. It is difficult to photograph, but it
can be shown to parents. In a case like this, it is
important to do a thorough eye examination and to
offer the family a plan to follow this infant.
293
Chapter 15
Measurements
40 pd ET - primary
AL0004
History Comment
6 month old boy The diagnosis of congenital esotropia is straight-
Vision: fixes and follows with both eyes forward. This infant fits the criteria for this diagno-
Refraction: OD +1.00 sis. The deviation is constant, 40 prism diopters and
OS +1.00 the child is over four months of age. The pictures
indicate the likelihood of cross fixation, but it is the
This six month old boy was noted by his parents examiners responsibility to demonstrate the infant's
to have crossed eyes beginning shortly after birth. willingness to take up fixation with either eye to rule
The pregnancy and delivery were normal. He is out amblyopia, and to confirm abduction in both eyes.
developing normally. The eye examination is normal At this time a V pattern (rarely an A) is looked for.
except for the esotropia. The presence of manifest or latent nystagmus is also
noted. Picture 7 shows fairly good abduction in the
right eye and picture 8 shows excellent abduction in
the left eye. These ductions are best demonstrated
with the doll's head maneuver (see p. 85). This child
is a candidate for surgery. In my hands this would be
a bimedial rectus recession putting the muscles back
10 to 10.5 mm from the limbus (or an appropriate
amount from the insertion).
294
Telemedicine: distance medicine
Measurements
20 pd ET - up
40 pd ET - primary
50 pd ET - down
ECA0022
History Comment
3 year old boy This boy has a V pattern congenital esotropia
Vision: fixes and follows well with both eyes with bilateral overaction of the inferior obliques and
Refraction: OD +1.50 +1.50 x 90 degrees underaction of the superior obliques, Also note the
OS +1.50 +1.50 x 90degrees antimongoloid fissures. This feature could be associ-
ated with pulley heterotopy. In this case the medial
The eye examination is normal except for signif- pulleys would be displaced upward and the lateral
icant fundus extorsion and the V esotropia with 4+ pulleys displaced downward. According to the pulley
overaction of the inferior obliques and significant theory, the V is caused by the vertical displacement
underaction of the superior obliques. of the horizontal rectus muscle action, relatively
weakening them in the direction of the displacement.
The V in this case would be treated with a medial
rectus recession with down shift. Pulley displace-
ment must be confirmed by coronal imaging of the
mid-orbit, but this is not readily available at this time
because of cost, availability, and patient cooperation.
The traditional treatment for a case like this is bime-
dial rectus recession with bilateral inferior oblique
weakening. However, some cases treated this way
have persistent overaction of the inferior obliques.
This could result from unrecognized, or untreated,
pulley displacement contributing to the vertical
incomitance. Combined inferior oblique weakening
and down shift of the recessed medial recti could also
be done. As an extreme, weakening of the inferior
obliques and tuck of the superior oblique, if they are
loose, could be done along with bimedial rectus
recession, but I think that is too much surgery for this
boy.
295
Chapter 15
Measurements
50 pd ET - up
60 pd ET - primary
70 pd ET - down
EC0024
History Comment
12 year old girl Of course, the amblyopia treatment should be
Vision: OD 20/200, OS 20/20 continued if at all possible. However, the odds of
Refraction: OD +.50 success are reduced as the child grows older for rea-
OS +.25 sons of cooperation if not for reasons related to the
amblyopia itself.
This 12 year old girl has deep amblyopia in the In order to avoid surgery on the better seeing eye
right eye that has been refractory to patching. A mild and still deal with the V pattern, this child could
V pattern is present with moderate overaction of the benefit from recession of the right medial rectus with
inferior obliques and underaction of the superior one half muscle width down shift and resection of the
obliques. Except for the strabismus and amblyopia right lateral rectus with one half muscle width up
the eye examination was normal including a normal shift. Another option would be bimedial rectus
fundus exam. recession with bilateral inferior oblique weakening,
but this would require surgery on the normal seeing
eye and is not a good idea. The recession and resec-
tion should both be maximum.
296
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Measurements
40 pd ET - up
40 pd ET - primary
60 pd ET - down
DR-Aybar0016
History Comment
4 year boy This pattern of V esotropia demonstrates under-
Vision: OD 20/40, OS 20/40 action of the superior obliques to a greater degree than
Refraction: OD + 3.00 the overaction of the inferior obliques. Pictures 3 and
OS + 3.00 (wears glasses but they have 5 show only mild strabismus surso-adductorius or
little effect on the angle) elevation in adduction. This boy has normal palpe-
bral fissure configuration suggesting that there is no
This boy was noted to have crossed eyes since pulley heterotopy. The superior oblique traction test
shortly after birth. His health is good and there is no may demonstrate a lax tendon. This would suggest
family history of strabismus. The eye examination that a superior oblique tuck along with a bimedial rec-
was normal except for the strabismus. The superior tus recession placing the medial recti 10 to 10.5 mm
obliques appear to underact to a greater degree than from the limbus or an appropriate distance from the
the inferior obliques overact. insertion would be appropriate. However, most sur-
geons would weaken the antagonist inferior obliques
even in the presence of a lax tendon.
297
Chapter 15
Measurements
Ortho - up
35 pd ET - primary
55 pd ET - down
DRD0021
History Comment
3 year old boy This boy has a V pattern which goes from ortho
Vision: OD poor fixation, OS fixes and follows in up gaze to 55 prism diopters of esotropia in down
Refraction: OD +2.00 gaze. The inferior oblique overaction and superior
OS +2.00 oblique underaction are apparent. With the ambly-
opia in the right eye note in picture 4 that the adduct-
This boy has amblyopia of the right eye V pat- ed left eye takes up fixation so that the strabismus sur-
tern, overaction of the inferior obliques, and underac- soadductorius or elevation in adduction is manifested
tion of the superior obliques. The remainder of the as a hypotropia of the abducted eye. The first order
eye examination is within normal limits. He also of treatment is to deal with the amblyopia. Then sur-
demonstrates chin depression preferring up gaze gical treatment could consist of weakening of the
where his eyes are aligned, this in spite of the ambly- inferior obliques along with a moderate medial rectus
opia in the right eye. recession with down shift. This down shift is because
of the likelihood of pulley heterotopy suspected
because of the slight antimongoloid fissure.
298
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Measurements
10 pd XT - up
20 pd ET - primary
45 pd ET - down
HAN0061
History Comment
13 year old girl At surgery this girl should have a careful superi-
Vision: OD 20/20, OS 20/30 or oblique traction test. If the tendons are loose or
Refraction: OD +1.50 +0.75 x 90 degrees lax, a bilateral superior oblique tuck could be done
OS +2.00 +0.50 x 90 degrees along with a moderate bimedial rectus recession mov-
ing the medial recti 8.5 mm from the limbus or an
This girl has very big overaction of the inferior appropriate amount from the insertion. For those not
obliques and underaction of the superior obliques. A inclined to do a superior oblique tuck, bilateral inferi-
moderate esotropia in the primary position actually or oblique weakening could be done. It is important
becomes and exodeviation in up gaze. The down in cases like this to perform the superior oblique trac-
gaze deviation is a larger esotropia. tion test even if you would have no intention of doing
a superior oblique tuck. Only by doing this test will
you get a feel for what is normal and what is abnor-
mal.
299
Chapter 15
Measurements
5 pd XT - up
30 pd ET (14 pd ET with Rx)
- primary
50 pd ET - down
RO - CHB0005
History Comment
6 year old girl Since the esodeviation is only 14 prism diopters
Vision: OD 20/25, OS 20/25 in the primary position with glasses, only a single
Refraction: OD +4.50 +1.00 x 100 degrees medial rectus should be recessed to 8.5 mm from the
OS +4.50 +1.00 x 80 degrees limbus or an appropriate amount from the insertion.
For the V pattern two options are possible. Either
This girl has an esotropia somewhat responsive the inferior obliques can be weakened or lax superior
to her hyperopic correction plus a V pattern with oblique tendons, if found at traction testing, can be
inferior oblique overaction superior oblique underac- tucked. In addition, the recessed medial rectus can be
tion and antimongoloid fissures suggestive of possi- displaced downward 14 muscle width and the other
ble pulley heterotopy. medial rectus also moved down 14 muscle width
without recessing it. Extreme V pattern with
evidence of pulley heterotopy suggests the possible
need for a double procedure to treat the V. This is
the type of case that could have persistent inferior
oblique overaction after proper weakening has been
done. The reason for this could be superior oblique
tendon laxity occurring on a congenital basis.
300
Telemedicine: distance medicine
Measurements
35 pd ET - up
20 pd ET - primary
55 pd ET - down
DRD0014
History Comment
11 year old girl This girl demonstrates vertical incomitance that
Vision: OD 20/20, OS 20/20 changes from esotropia in primary position and
Refraction: OD +1.00 upgaze to large angle exotropia in down gaze. This
O S +1.00 is present along with pronounced mongoloid fissures.
The exotropia in down gaze seems to be caused by the
This girl demonstrates an A pattern with an exaggerated abducting action of the superior oblique
esotropia in upgaze and a large exotropia in muscles. This behavior is likely to be caused by
downgaze. Very evident mongoloid fissures are also downward displacement of the medial pulleys and
present. What is usually called overaction of the upward displacement of the lateral pulleys as would
superior obliques is also present be expected to occur with a mongoloid fissure.
Treatment in this case could be:
1) Recession of both medial rectus muscles to
10.0 mm from the limbus or an appropriate
amount from the insertion with 1/2 muscle
width upshift
2) Recession of both lateral rectus muscles 5.0
mm with 1/2 muscle width downshift
Some surgeons might choose bilateral superior
oblique weakening with tenectomy or recession com-
bined with a bimedial rectus recession with or without
upshift
301
Chapter 15
Measurements
60 pd ET - up
50 pd ET 25 pd R hyper -
primary
20 pd ET - down
DRR0015
History Comment
23 year old female A surgical plan for this woman could avoid sur-
Vision: OD 20/50, OS 20/20 gery on the oblique muscles and include the follow-
Refraction: OD plano -1.50 x 180 degrees ing:
OS +1.00 -1.00 x 180 degrees 1) Right eye: recess the medial to 10.0 mm from
the limbus or an equivalent from the insertion
This woman has had an esotropia since birth. with 1/2 muscle width upshift and recess the
She has a mild amblyopia in the right eye, and a right superior rectus 5.0 mm
hypertropia. The oblique muscles do not overact and 2) Left eye: recess the medial rectus 10.0 mm
the palpebral fissures are normal. from the limbus or an equivalent amount from
the insertion with 1/2 muscle width upshift
and resect the lateral rectus 6.0 mm with 1/2
muscle width downshift.
Even though there is no fissure obliquity sug-
gesting pulley heterotopy the fact that the obliques do
not appear to be overacting suggests that vertical dis-
placement of the horizontal recti would be best.
302
Telemedicine: distance medicine
Measurements
80 pd ET - up
75 pd ET - primary
40 pd ET - down
DRR0017
History Comment
11 year old boy Treatment of this A pattern could consist of the
Vision: OD 20/20, OS 20/20 following:
Refraction: OD +1.00 -1.50 x 180 degrees 1) Bimedial rectus recession to 11.0 mm from
O S +1.50 -0.75 x 180 degrees the limbus or equivalent distance from the
insertion with 1/2 muscle width upshift
This boy developed an esotropia about age 5 2) Bilateral lateral rectus resection 5 or 6 mm
years according to his parents. The A pattern is pres- with 1/2 muscle width downshift
ent without apparent superior oblique overaction or The patient should be tested for optokinetic
inferior oblique underaction. There is no explanation asymmetry to determine if the likely etiology is con-
why the deviation started so late. genital esotropia
303
Chapter 15
Measurements
40 pd ET - up
30 pd ET - primary
20 pd E(T) - down
ECA0026
History Comment
34 year old male A logical treatment plan for this man includes:
Vision: OD 20/20, OS 20/30 1) Bimedial rectus recession 11.0 mm from the
Refraction: OD -0.50 -0.50 x 171 degrees limbus or equivalent amount measured from
OS plano -0.75 x 180 degrees the insertion
Fuses: Worth four-lights in down gaze; no stereopsis 2) Upshift of the recessed medial rectus muscles
measured 1/2 to 3/4 muscle width.
Postoperative pictures show the man with an
This man suffers from chronic neck ache from improved head posture.
holding his chin up and looking down to avoid
diplopia. He has an A pattern esotropia. He avoids
diplopia with the head posture and even demonstrates
some fusion ability in down gaze. To avoid double
vision he pays the price of physical discomfort.
304
Telemedicine: distance medicine
Measurements
60 pd XT - up
60 pd XT - primary
60 pd XT - down
DRR0018
History Comment
10 month old boy Treatment of congenital exotropia in this case
Vision: fixes and follows well with each eye can be managed with a bilateral lateral rectus reces-
Refraction: OD +1.50 sion of 7.0 mm. To this could be added a moderate
O S +1.50 resection of one medial rectus.
305
Chapter 15
Measurements
90 pd XT - up
70 pd XT - primary
50 pd XT - down
DRR0034
History Comment
3 year old boy This large angle V pattern congenital exotropia
Vision: fixes and follows well with both eyes could be treated with the following:
Refraction: OD +1.00 -2.00 x 90 degrees 1) Bilateral lateral rectus recession 7.5 mm
O S +1.00 -0.50 x 90 degrees 2) Resect one medial rectus muscle 5.0 mm
3) Bilateral inferior oblique weakening
The family noted an exodeviation at 3 months. The
childs general health is good and the eye examina- The recessed lateral rectus muscles could also be
tion is normal except for the V pattern exotropia. shifted up 1/2 muscle width and the resected medial
There is significant overaction of the inferior rectus muscle shifted down 1/2 muscle width and the
obliques. inferior oblique weakening omitted.
306
Telemedicine: distance medicine
Measurements
70 pd XT - up
50 pd XT - primary
50 pd XT - down
DRR0013
History Comment
5 year old boy This may be an example of an intermittent exotropia
Vision: OD 20/20, OS 20/20 progressing to a constant exotropia over time. Some
Refraction: OD plano -0.75 x 180 surgeons feel strongly about operating earlier on
OS plano -0.75 x 180 exodeviations in infants and toddlers to avoid this.
The surgical treatment for this would be:
This 5 year old boy started with an intermittent 1) Large bilateral lateral rectus recession 7.0 to 8.0
exotropia which gradually became constant. There is mm with 1/4 muscle width up shift - or
moderate overaction of the inferior obliques with a 2) Recession one lateral rectus 7.0 to 8.0 mm with 1/4
V pattern. The remainder of the eye examination is muscle width upshift and resection one medial rec-
within normal limits. The child is otherwise healthy. tus 7.0 mm with 1/4 muscle width down shift.
307
Chapter 15
Measurements
30 pd X(T) - up
20 pd X(T) - primary
15 pd X(T) - down
AL0014
History Comment
13 year old girl This is a straightforward case of intermittent
Vision: OD 20/20, OS 20/20 (with correction) exotropia. There is no urgent need for surgery, but on
Refraction: OD -3.00 -1.00 x 165 degrees the other hand it could be done at any time. A bilat-
OS -1.00 2.00 x 20 degrees eral lateral rectus recession of 5.0 to 6.0 mm with 1/2
Fuses: 9/9 stereo (40 sec.) muscle width up shift would be sufficient. In the
event that surgery is not done at this time, regular fol-
This girl has been noted by her parents and her low up at no longer than 6 month intervals is recom-
friends to have an eye that wanders out when she is mended.
tired or when she is day dreaming. She habitually
closes one in bright sunlight. The girl has no aware-
ness of this unless it is called to her attention. She is
symptom free.
308
Telemedicine: distance medicine
Measurements
25 pd XT - primary
ECX0032
History Comment
16 month old girl This child will benefit from surgery. This could
Vision: fixes and follows well with both eyes be a moderate bilateral lateral rectus recession with
Refraction: OD + 2.00 the muscles recessed 5.0 or 6.0 mm with a 1/2 muscle
OS + 2.00 width upshift to treat the V pattern. It is very possi-
ble that this treatment could result in normal or near
This child was brought to the ophthalmologist at normal binocularity if this deviation did begin as an
age 10 months with the complaint that one eye was intermittent exotropia.
drifting outward. Pictures from the newborn nursery
show that the eyes appeared to be aligned or possibly
converged.
309
Chapter 15
Measurements
ortho - up
20 pd XT - primary
45 pd XT - down
ECA0023
History Comment
11 year old girl Study of these pictures reveals some interesting
Vision: OD 20/20, OS 20/20 findings. The motility looks normal in pictures 1-6
Refraction: OD plano +2.75 x 65 degrees with a small XT in the primary position, right and left
OS plano +3.50 x 115degrees gaze and eyes nearly aligned in up gaze. However in
Stereo: nil pictures 7-8-9 we see overaction of the superior
obliques and a big A pattern. Added to this in pic-
This girl had a bimedial rectus recession for con- tures 10 and 11 we see DVD. This is the triad: A
genital esotropia at age 9 months. At age 3 years the pattern, overaction of the superior obliques , and
mother noticed the eyes beginning to deviate mostly DVD. In this case I believe the DVD is a carry over
outward. of congenital ET and the A pattern is due to the
superior obliques abducting action influenced by the
retroplacement of the medial recti. Surgical options
for this patient include: 1) bilateral superior rectus
recession for the DVD 2) advancement of one medial
rectus for the primary position XT and to reduce the
down gaze XT by altering the Superior oblique
action. As an alternative a small recession of one or
both lateral rectus muscles with downshift 1/2 muscle
width could be done. In this case I think it is impor-
tant to avoid the temptation of doing a bilateral supe-
rior oblique weakening procedure. I say this because
I believe that the superior obliques are allowed to
manifest their abducting action because of the posi-
tion of the globe resulting from the medial rectus
recession.
310
Telemedicine: distance medicine
Measurements
95 pd XT - up
45 pd XT - primary
ortho - down
DRR0045
History Comment
17 year old female Surgery in this case could included the follow-
Vision: OD 20/20, OS 20/20 ing:
Refraction: OD +0.75 1) Bilateral lateral rectus recession 7.0 or 8.0 mm
OS +1.00 with 1/2 to one full muscle width upshift
2) Resection one medial rectus 8.0 mm with 1/2
This patient has a huge V pattern with what we muscle width downshift
call overaction of the inferior obliques. Note in pic- 3) Bilateral inferior oblique weakening (myecto-
tures 3 and 5 that adduction is limited and that in lat- my or recession)
eroversions the eyes move up and abduct as though As with any case where pulley heterotopy is sus-
the inferior obliques were exerting their seconday pected, coronal imaging of the orbit with CT or MRI
action of abduction. In addition there is a moderate would aid in the diagnosis and plan, but expense and
antimongoloid fissure suggesting that the medial pul- availability make this impractical at this time.
leys may be dislocated upward and the lateral pulleys
downward! In spite of having eyes that are aligned
in far down gaze, this patient demonstrates no fusion
and has no diplopia. No prior surgery had been done.
311
Chapter 15
Measurements
80 pd XT - up
50 pd XT - primary
75 pd XT - down
DRR0051
History Comment
14 year old girl With an X pattern like this only horizontal sur-
Vision: OD 20/20, OS 20/20 gery is required. There is no need to weaken all of the
Refraction: OD plano obliques as has been suggested. Surgery could
OS plano include the following:
Stereo: nil 1) Bilateral lateral rectus recession 7.5 mm
2) Resection of one medial rectus 6.0 mm
This girl has had an exotropia since age 1 year. If the surgeon chooses, one of the muscles could
The angle in the primary position is large, 50 prism be placed on an adjustable suture. If this were done,
diopters but it is even larger in both up and down I would elect the lateral rectus in the eye not having
gaze. Note that in pictures 3 and 5 there is no appar- the medial rectus resection.
ent elevation in adduction, the usual sign of oblique
overaction. The eyes take off while moving up or
down demonstrating the secondary abducting action
of both the inferior and superior obliques creating
what is called an X pattern.
312
Telemedicine: distance medicine
Measurements
35 pd XT - up
30 pd XT - primary
35 pd XT - down
DR-Aybar0013
History Comment
6 year old girl Because of the poor vision in the left eye, surgery
Vision: OD 20/70, OS counts fingers for the exotropia should be limited to this eye. A
Refraction: OD +2.50 -3.00 x 180 degrees recession of the left lateral rectus muscle of 7.5 mm
OS -10.00 sph. and a resection of the left medial rectus of 5.0 mm
would be safe and not likely to produce an over cor-
This girl has a dense amblyopia in the left eye rection.
probably because of the high anisomyopia. It would
be a good idea to check the axial length of the left eye
and also to take a careful look at the retina. This girl
could develop the heavy eye complication of high
myopia in later years. I do not know a way of keep-
ing this from happening.
313
Chapter 15
Measurements
70 pd XT - up
60 pd XT - primary
95 pd XT - down
20 pd L hyper OD fix
30 pd R hyper OS fix
DRR0036
History Comment
34 year old female This is a large angle with, an X pattern and the
Vision: OD 20/25, OS 20/70 patient also has DVD. The DVD is manifest and the
Refraction: OD -0.5 -0.50 x 90 degrees patient states that this bothers her. A possible surgical
OS -0.50 sph approach is the following:
1) Bilateral lateral recession 8.0 mm
This woman has had a life long exotropia which 2) Vessel sparing tuck of the left medial rectus
has increased in the past 5 years. She would like to (6.0 mm)
improve her appearance and is eager to have surgical 3) Bilateral superior rectus recession 6 mm OD
correction for the exodeviation. and 5.0 mm OS)
The medial rectus tuck is suggested to avoid sev-
ering the anterior ciliary vessels of three rectus mus-
cles in one eye.(see p. 211) An adjustable suture
could also be added to the recession of the left lateral
rectus.
314
Telemedicine: distance medicine
Measurements
20 pd XT - up
5 pd XT - primary
25 pd XT - down
DVD both eyes
OS >> than OD
DR0003
History Comment
5 year old boy This patient presents a challenge when it comes
Vision: OD 20/25, OS 20/25 to arriving at a plan for surgery. The primary position
Refraction: OD +2.00 sph deviation is small, hardly noticeable and the DVD
OS +2.00 sph seems to be manifest in only one eye with the other
eye never being seen up except under cover and then
This child has had an esodeviation noted by the only a small amount. A logical plan would be:
parents since about for months of age. He demon- 1) Move both medial rectus muscles up 3/4 mus-
strates a mild A pattern and has a left hyperdeviation cle width without recession (being careful to
that behaves like dissociated vertical deviation offset any resection effect resulting from
(DVD). The right eye is said to also demonstrate a suture placement) or move both lateral recti
hyperdeviation but only under cover, a small amount, down taking the same precaution.
and with a rapid recovery when the cover is removed. 2) Recess the left superior rectus 4.0 to 5.0 mm.
This could not be captured with a picture. In contrast, Watch for a manifest hyperdeviation from the DVD
the left hyperdeviation is becoming manifest more occurring later in the right eye.
often according to the parents.
315
Chapter 15
Measurements
ortho - up
ortho - primary
50 pd XT - down
GUM0039
History Comment
9 year old boy A patient like this who has some fusion but who
Vision: OD 20/30, OS 20/30 is very troubled by an A pattern with overaction of
Refraction: OD +2.25 -1.00 x 180 degrees the superior obliques raises the question, is it safe to
OS +1.75 -1.25 x 180 degrees weaken the superior obliques in a fusing patient?.
Fuses: stereo fly (3,000 sec.) This can be done, but it could be risky because it
is difficult to perform symmetrical weakening of the
This boy is having difficulty in school with read- superior oblique. There is always the chance of cre-
ing and doing work at the board. His handwriting is ating a postoperative vertical deviation that would
said to be terrible. The parents deny any abnormal spoil fusion. In this case the two choices for surgery
head posture. He was not observed at this examina- are;
tion to assume a chin down posture that would be 1) Down shift of the lateral recti without reces-
expected in this type of strabismus. There is what is sion
described as overaction of the superior obliques. The 2) Bilateral weakening of the superior obliques
boy is otherwise healthy and there is no family histo- Another option that I have not done is bilateral nasal-
ry of strabismus. ward shift of the inferior rectus muscles.
This case differs rom case 59 in chapter 16 in that
the boy is having a great deal of trouble in school.
316
Telemedicine: distance medicine
Post op.
Measurements
post op primary 35 pd ET
Pre op primary 45 pd XT
Pre op.
ROL0059
History Comment
42 year old male The surgery for the consecutive exotropia avoid-
Vision: OD 20/30, OS 20/30 ed the medial recti because of what was thought to be
Refraction: OD +1.25 thin sclera. This surgery for the consecutive esotropia
OS +0.75 could do likewise. A surgical option for this case
would be advancement of the of previously resected
This man has a history of what appears to be con- and later recessed lateral recti. This advancement
genital esotropia. He had a bimedial rectus recession could be done with a tandem adjustable suture placed
at age 22. Because of a residual esotropia he later on one of the advanced muscles. My recommenda-
underwent bilateral lateral rectus resection. This tion is that the lateral rectus muscles be advanced 6.0
resulted in a large angle consecutive exotropia. or 7.0 mm. With the tandem adjustable suture, the
Because of apparent thin sclera medially a bilateral muscle would be strengthened the maximum
lateral rectus recession (of the previously resected amount that could be anticipated. The second or tan-
muscles) was done. The right lateral rectus was dem suture could be used to weaken the muscle by
recessed 10.0 mm and the left lateral rectus was hanging it back if needed.
recessed 7.0 mm. This resulted in 35 prism diopters
of esotropia. The patient (and the surgeon) are eager
to have the eyes aligned.
317
Chapter 15
Measurements
30 pd XT, 10 L hyper - up
35 pd XT 25 L hyper -
primary
55 pd XT 25 L hyper - down
DR-Aybar0003
History Comment
42 year old female Aligning a non-seeing eye is a legitimate under-
Vision: OD 20/20, OD light perception taking using the dictum every humans has the right
Refraction: OD plano to look like a human. Surgery should be restricted
OS +8.50 whenever possible to the poorer seeing eye. In this
case it is wise to avoid the left superior rectus
This woman had a traumatic cataract of the left because of concerns about thin retina as a result of
eye removed as a child. She never regained good prior tumor surgery in the area. A logical choice for
vision. Shortly after she had an intraocular tumor surgery would be:
removed from the superior aspect of the left eye. 1) Recession of the left lateral rectus 8.0 mm
Now she has an exotropia and hypertropia of the left with 1/2 muscle width down shift
eye. She would like to have her eyes straightened. 2) Resection of the left medial rectus 8.0 mm
with 1/2 muscle width downshift
3) Left inferior oblique weakening (myectomy
or recession)
The downshift of both of the horizontal recti in
the left eye will have some effect on lowering the
hyper deviated left eye. The inferior oblique is not the
most effective muscle to weaken for treating this kind
of hyperdeviation, but there is very little downside in
this case. If needed a left levator resection could be
done after the results of the muscle surgery are
known.
318
Telemedicine: distance medicine
Measurements
60 pd XT - up
70 pd XT - primary
95 pd XT - down
DRR0035
History Comment
29 year old male The obvious mongoloid fissures suggest the pos-
Vision: OD 20/25, OD 20/20 sibility of pulley heterotopy contributing to the A
Refraction: OD +2.00 -1.50 x 15 pattern. Disregarding the first surgery, at least for
OS +1.50 -1.25 x 180 now, a logical surgical plan would be the following:
1) Recess both lateral rectus muscles 8.0 mm
This man has a life long history of exotropia. He with 1/2 muscle width downshift
had surgery for this at age 3 years but there is no 2) Resect both medial recti 8.0 mm width 1/2
record of what was done. He would like to have his muscle width upshift.
eyes straightened now. He has no double vision or One of the lateral rectus muscles could be placed
other visual symptoms. on an adjustable suture.
319
Chapter 15
Measurements
60 pd XT - up
45 pd XT - primary
65 pd XT - down
ROD0047
History Comment
27 year old male The best option for surgery in a case like this is
Vision: OD 20/25, OS 20/30 advancement and resection of the previously recessed
Refraction: OD plano +0.75 x 90 degrees medial rectus muscles. This presents the challenge of
OS plano +0.50 x 80 degrees finding the muscles that in this case are likely to be
easily found because adduction is present though
This man had surgery for congenial esotropia at diminished. When advancing previously recessed
age 4 years according to history obtained from the muscles, it is the rule to find them stiff making it
patient. Scars over the medial aspect of the globe impossible to pull them up to the original insertion.
support this. He denies double vision. Adduction is Given this, it is useful to combine resection with
limited in both eyes, more so in the right. The patient advancement arriving at a surgical number that is a
would like to have his eyes aligned and is eager for combination of the two. In this case; for example, 8.0
surgery. mm of surgery on each medial rectus muscle could
mean a 5 mm advancement and a 3.0 mm resection.
In this case it might be prudent to place one of the
muscles on an adjustable suture.
320
Telemedicine: distance medicine
Measurements
60 pd XT and
15 pd R hypo -
primary position
HAN0148
History Comment
30 year old male This case demonstrates complete third nerve
Vision: OD 20/20, OS 20/20 palsy with pupil sparing and without aberrant regen-
Refraction: OD plano eration. The only extraocular muscles functioning in
OS plano the right eye are the lateral rectus and the superior
oblique. The action of these muscles drives the eye
This man sustained closed head injury in an auto downward with incycloduction and out. Surgical
accident 9 months earlier. He was comatose for 2 treatment would include:
days. His motility has remained stable since that time 1) Large recession of the right lateral rectus of
with a large right exotropia and hypotropia, with pto- 10+mm, disinsertion, or attachment to the lat-
sis of the right upper lid. The right pupil responds to eral periostium
light and accommodation. When he raises his lid, he 2) Transposition of the right superior oblique to
sees double. The Bell phenomenon is absent. the superior border of the insertion of the right
medial rectus holding the eye in slight adduc-
tion and done without fracture of the trochlea.
3) Brow suspension of the right upper lid, under-
correcting to avoid corneal exposure.
321
Chapter 15
Measurements
35 pd XT
15 pd L Hypo
HAN0172
History Comment
11 year old girl An attempt at patching the right eye for treatment
Vision: OD 20/20, OS 20/200 of the amblyopia would be a good place to start. This
Refraction: OD plano would require that the girl assume a chin up and face
OS plano turn to the right. However, this treatment has a possi-
ble downside. At present she has no diplopia. If
This girl has had the left eye down and out with amblyopia treatment improves vision in the left eye
ptosis since birth. The pupil in the left eye remains sufficiently to make it difficult to suppress, then the
reactive. A dense amblyopia is present in the left eye. patient will be bothered by diplopia when her eyes are
No prior treatment for the amblyopia had been given. better aligned and the left upper lid is raised at sur-
The lids are being held up to show motility in down gery.
gaze. No aberrant regeneration of the levator or the Surgery in this case would be:
other extraocular muscle is seen. 1) Maximum recession of the left lateral rectus
2) Transfer of the left superior oblique tendon to
the superior border of the left medial rectus
without fracture of the trochlea.
3) frontalis suspension of the left upper lid in a
slightly under corrected position to protect the
left cornea.
322
Telemedicine: distance medicine
Measurements
45 pd XT
5 R hypo primary
ROL0089
History Comment
30 year old female In this case of partial third nerve palsy without
Vision: OD 20/25, OS 20/20 aberrant regeneration, there appears to be some func-
Refraction: OD plano -1.50 x 10 tion of the right medial rectus. For this reason the fol-
OS plano -1.50 x 10 lowing could be reasonable surgical option:
1) Large recession of the right lateral rectus
This woman suffered a cerebral hemorrhage 2) Large resection of the right medial rectus
when giving birth one year ago. Her right eye is 3) Tenectomy of the right superior oblique
down and out with evidence of only the right lateral In addition both the medial and lateral rectus mus-
and right superior oblique muscles functioning. The cles could be shifted up 1/2 muscle width.
right pupil is slightly dilated, but the right levator
palpebri seems to function normally. This woman is
bothered by constant diplopia. She has had no other
signs or symptoms from the apparent stroke. There
is no evidence of aberrant regeneration.
323
Chapter 15
Measurements
80 pd XT
HANOI0035
History Comment
20year old male With absent adduction, elevation, and depression
Vision: OD 20/20, OS 20/20 even without lid and pupil involvement, this appears
Refraction: OD -0.50 to be bilateral congenital third nerve palsy. However,
O S -0.75 since there appears to be telecanthus present, the inter
canthal distance should be measured. If this distance
This man was presented for consultation with a is more than 1/2 the value of the pupillary distance,
presumptive diagnosis of bilateral third nerve palsy telecanthus with the strong possibility of a midline
with sparing of the lids and pupils. defect can be suspected. Imaging with a CT scan
should be obtained. If a midline defect were seen, it
would not necessarily change the treatment, but it is
information that should be known.
Surgery in this case would be large bilateral lat-
eral rectus recession and bilateral superior oblique
tendon transfer to the medial rectus insertion without
trochlear fracture. However this would depend on
findings after additional work up that could possibly
demonstrate missing muscles and more.
324
Telemedicine: distance medicine
Measurements
50 pd XT
20 L Hypo
RO0042
History Comment
14 year old girl This is another example of third nerve palsy with
Vision: OD20/20, OS 20/40 the typical findings of the affected eye being down
Refraction: OD -0.50 and out with ptosis and in this case a dilated pupil.
OS -1.25 This girl is fortunate to have both good vision in the
involved eye and no diplopia. As in other cases
This girl suffered encephalitis at age 1 year. described, this girl would benefit from a large left lat-
After that she developed a left third nerve palsy that eral rectus recession, transfer of the left superior
has remained unchanged until the present. She denies oblique to the medial rectus insertion without fracture
diplopia, but has retained good vision in the left eye. of the trochlea, and frontalis suspension of the left
upper lid with a slight under correction to protect the
cornea.
325
Chapter 15
Measurements
10 pd ET
15 R hypo primary
DRR0070
History Comment
34 year old male This case will definitely benefit from a CT scan
Vision: OD 20/20, OS 20/15 of the orbit. This is necessary to determine whether
Refraction: OD plano +1.25 x 90 degrees or not a blowout fracture is present. This could
OS plano +1.25 x 90 degrees account for the limited elevation. There also remains
the possibility of there being two problems, a partial
This man suffered severe head trauma in a motor third nerve palsy and a blowout fracture of the right
vehicle accident. He presents with a right eye that is orbit. No treatment plan can be formulated until fur-
down, but not necessarily out! Some of the features ther evaluation is completed.
of a third nerve palsy are present. The right eye is
hypotropic, elevation is restricted, there is some pto-
sis, and the right pupil is dilated. Also there appears
to be some lid elevation of the right eye on down
gaze. However the right eye adducts well, and there
is more depression than can be attributed to the supe-
rior oblique acting as the sole right eye depressor.
326
Telemedicine: distance medicine
AL0012
History Comment
11 year old female In cases of class I superior oblique palsy with a
Vision: OD 20/20, OS 20/20 maximum hyperdeviation of no greater than 25 prism
Refraction: OD +1.50 diopters weakening of the antagonist inferior oblique
OS +1.50 is effective. The left hyperdeviation in left head tilt,
Motility: picture 11 (positive Bielschowsky test) is difficult to
appreciate in the pictures because the patient is fixing
with the left eye resulting in a less noticable right
25 LHT* 10 LHT* 8 LHT* hypotropia with the lid following the globe to further
obscure the difference in the level of the eyes.
20 LHT 15 LHT 10 LHT
327
Chapter 15
HAN0014
History Comment
30 year old male This man began noticing occasional diplopia one
Vision: OD 20/20, OS 20/20 year ago. He denies any trauma. His health is good
Refraction: OD plano and his eye examination is otherwise normal. The
OS plano most significant motility feature is underaction of the
Motility: left superior oblique. The greatest vertical deviation is
in the field of action of the paretic superior oblique.
This results in a Class II superior oblique palsy. If at
5 LHT 5 LHT 0 surgery a lax left superior oblique tendon were found,
a small tuck could be done. If the superior oblique
15 LHT 20 LHT 5 LHT traction test were normal, a weakening of the yoke to
the left superior oblique, the right inferior rectus
20 LHT 20 LHT 20 LHT could be effective.
328
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AL0024
a History Comment
15 year old male This patient had significant head trauma one year
Vision: OD 20/20, OS 20/20 ago with a brief period of loss of consciousness. After
Refraction: OD +1.00 that he noticed frequent double vision and increasing
OS +.75 (no glasses worn) head tilt. His condition has remained stable for the
Motility: last six months. With a vertical deviation largest in
the fields opposite the paretic eye; that is, in left gaze
in the case of a right superior oblique palsy, a Class
10 RHT 16 RHT 30 RHT III superior oblique palsy is diagnosed. Since the
deviation is greater than 25 prism diopters in the field
8 RHT 18 RHT 36 RHT of greatest deviation two vertical muscles need to be
treated. In this case the right inferior oblique would
10 RHT 16 RHT 25 RHT be weakened along with either a right superior
oblique tuck (very unlikely in the case of an acquired
superior oblique palsy because the tendon is expected
Right Tilt 30 RHT to be normal) or a left inferior rectus recession. The
latter being the yoke of the paretic right superior
Left Tilt 8 RHT oblique.
329
Chapter 15
Congenital absence
of the left
superior oblique
ECA0020
History Comment
9 year old male This nine year old male had a life long history of
Vision: OD 20/20, OS 20/20 a large right head tilt. He also was noted by his fam-
Refraction: OD plano +.75 x 90 degrees ily to have a much fuller face on the left. The size of
OS plano the deviation, the facial asymmetry and the pro-
nounced head tilt led to the suspicion that there may
Motility: be an absent left superior oblique tendon. A coronal
CT confirmed this. Note that the superior oblique is
seen in the right orbit but not the left. This finding
20 LHT* 15 LHT 10 LHT was later confirmed at surgery. The pattern of the
deviation indicates a Class III or possibly IVsuperior
40 LHT 30 LHT 23 LHT oblique palsy. Because there is no superior oblique to
strengthen on the left, surgery for this deviation
35 LHT 35 LHT 25 LHT
would consist of weakening the left inferior oblique,
and recession of the right inferior rectus with the pos-
* A small ET was measured in all fields sible addition of a small to moderate left superior rec-
tus recession.
Right Tilt 0
330
Telemedicine: distance medicine
AL0008
History Comment
12 year old female With maximum deviation greater than 25 prism
Vision: OD 20/20, OS 20/35 diopters and the larger deviations in the field of both
Refraction: OD + 2.00 the paretic superior oblique and the antagonist inferi-
OS + 2.00 or oblique this patient has a class III superior oblique
Motility: palsy requiring two muscle surgery. The left inferior
oblique would be weakened along with a tuck of the
left superior oblique if the tendon were found to be
28 LHT* 12 LHT 12 LHT loose on traction testing or right inferior rectus weak-
ening if the left superior traction test were normal.
25 LHT 18 LHT 6 LHT
* A small XT in upgaze
331
Chapter 15
CR - HNN0004
History Comment
6 year old male The pattern of this deviation is that the larger left
Vision: OD 20/30, OS 20/30 hyper is in the entire right field plus a large deviation
Refraction: OD +1.00 +.50 x 90 is present in down left gaze. This latter is due to con-
OS +1.00 +.50 x 90 (no glasses worn) traction of the left superior rectus which is the yoke of
the right inferior oblique which is the antagonist to
Motility: the paretic right superior oblique. In this case the
deviation is treated by weakening the left inferior
20 LHT 10 LHT 8 LHT oblique to treat the deviation in right gaze plus a
recession of the left superior rectus to deal with the
20 LHT 28 LHT 25 LHT hyperdeviation in the down left field. This pattern of
superior oblique palsy was first described by
22 LHT 20 LHT 26 LHT Jampolsky who pointed out that the contracted supe-
rior rectus created fixation duress in the antagonist
ipsilateral inferior rectus which by Herings law influ-
ences the normal superior oblique to overact. The
Right Tilt 4 LHT important thing to remember in this pattern of superi-
or oblique palsy is that the overacting superior
Left Tilt 25 LHT oblique should not be weakened!
332
Telemedicine: distance medicine
ECA0017
OD OS - extorted globe
History Comment
45 year old female This patient began noticing occasional diplopia
Vision: OD 20/20, OS 20/25 over the past two years. She also has developed a
Refraction: OD -.25 +.25 x 95 degrees chronic left head tilt. She fixates with her paretic right
OS -.50 +.25 x 75 (no glasses worn) eye. This appears to be producing fundus torsion
expressed as excyclotorsion of the left fundus. With
Motility: the greater vertical deviation in the lower fields, this
is closest to a Class V superior oblique palsy. It could
be treated with a right superior rectus recession and a
4 RHT 6 RHT 12 RHT recession of the left inferior rectus could be added.
As an alternative, an anterior and lateral shift of the
15 RHT 20 RHT 22 RHT the anterior fibers of the right superior oblique could
be done with a right superior rectus recession.
20 RHT 20 RHT 20 RHT Treatment is aimed at dealing with a hypertropia in
both the right and left fields of down gaze. Also with
no hypertropia in left head tilt and significant torsion
Right Tilt 28 RHT it is a good idea to be watching for a masked bilat-
eral superior oblique palsy.
Left Tilt 0
333
Chapter 15
HAN0018
History Comment
9 year old male This patient demonstrates a pattern of hyper-
Vision: OD 20/20, OS 20/50 tropia with the largest deviation measured on down
Refraction: OD -.75 gaze or as it has been referred to across the bottom.
OS -5.00 +3.50 x 65 degrees This is called a Class V superior oblique palsy.
Motility: Effective treatment of this class of deviation starts
with recession of the ipsilateral superior rectus that in
this case is the right superior rectus. Then either tuck
4 RHT 0 0 of a loose superior oblique tendon, on the right in this
case, or if the paretic superior oblique has a normal
8 RHT 10 RHT 6 RHT tendon, a recession of the yoke left inferior rectus.
Left Tilt 0
334
Telemedicine: distance medicine
CASE 43: Class III superior oblique palsy with pseudo ptosis
(inhibitional palsy of the contralateral antagonist)
HAN0049
History Comment
14 year old female The special feature of this case is that the patient
Vision: OD 20/20, OS 20/20 appears to prefer the left eye or the eye with the paret-
Refraction: OD plano ic superior oblique for fixation. This results in more
OS plano innervation to the paretic left superior oblique and by
Motility: Herings law the same robust innervation to the nor-
mal yoke, the right inferior rectus. This in turn caus-
es excess inhibition to the antagonist of this yoke
20 LHT 20 LHT 10 LHT muscle which is the right superior rectus. This inner-
vation level also dictates the innervation to the right
18 LHT 16 LHT 6 LHT levator palbebri. Since this antogonist gets less inner-
vation, a ptosis (or more correctly a pseudo ptosis) is
16 LHT 8 LHT 4 LHT present. This is called inhibitional palsy of the con-
tralateral antagonist. The important lesson to
remember is that fixing the motility defect automati-
Right Tilt 0 cally fixes the ptosis.
335
Chapter 15
HAN0085 HAN0114
History Comment
9 year old male The preoperative motility points to a right supe-
Vision: OD 20/25, OS 20/25 rior oblique palsy with a rather pronounced left head
Refraction: OD plano tilt as would be expected. In addition some facial
OS plano asymmetry is present with the fuller face on the side
Fusion: stereo acuity 40 seconds of the paretic superior oblique, a finding with the
Motility: rule. Before the patient underwent surgery a question
about the role of tight neck muscles on the head pos-
Pre-operative
ture was discussed and correctly dismissed. A weak-
ening procedure of the right inferior oblique was
done.
20 RHT The postoperative appearance of the patient
shows essentially normal alignment. Only a slightly
positive Bielschowsky test remains as evidenced by a
small residual right hypertropia in right head tilt. But
most important, the head posture is now normal. This
confirms that the anomalous head posture was due to
the vertical deviation and not due to a tight neck mus-
cle.
336
Telemedicine: distance medicine
RO0023
History Comment
10 year old female This girl demonstrates a typical class III left
Vision: OD 20/20, OS 20/20 superior oblique palsy with the expected right head
Refraction: OD +1.00 tilt. Her parents state that she has always demonstrat-
OS +1.00 ed this head tilt. Her general health is excellent and
Fusion: stereo acuity 40 seconds her eye examination is otherwise normal. This is
Motility: apparently a congenital superior oblique palsy, but
this girl does not demonstrate facial asymmetry. She
does on direct questioning admit to occasionally see-
25 LHT 10 LHT 5 LHT ing images doubled vertically, but she can fuse the
images readily. In a case like this with a hypertropia
25 LHT 15 LHT 4 LHT measuring 25 prism diopters at the maximum, that is
in up right gaze, the patient can be treated effectively
20 LHT 5 LHT 0 with a weakening of the left inferior oblique. A sec-
ond surgery could be needed for an undercorrection
many years in the future.
337
Chapter 15
ROL0006
History Comment
4 year old female A striking feature in this child is the facial asym-
Vision: Fixes and follows OU, no cooperation for metry with a much larger cheek on the right side, the
optotypes side of the paretic superior oblique. The esotropia
Refraction: OD +3.00 +1.00 x 10 degrees suggests that this child does not have fusion and
OS +3.50 +.75 x 90 degrees (wearing although a moderate left head tilt is present, it does
glasses) not compare to the facial asymmetry. In a case like
Motility: this it is especially important to determine the status
of the superior oblique tendon. This is the type of
5 RHT* 12 RHT 30 RHT patient where the tendon may be absent or extremely
loose. This can be determined by a careful superior
* A variable esotropia +/- 12 oblique traction test and confirmed by exploration of
the superior oblique tendon at the time of surgery.
Double maddox rod: unable Note in picture 3 the right hypertropia is manifested
as a left hypotropia because the right eye is fixing.
338
Telemedicine: distance medicine
Measurements
70 pd ET - up
80 pd ET - primary
90 pd ET - down
HAN0111
History Comment
46 year old female Treatment in this case would consist of:
Vision: OD 20/25, OS 20/40 1) Bilateral transfer of the vertical rectus muscles
Refraction: OD plano to the lateral rectus muscle. The question
OS plano would be, full tendon or half tendon? With or
without augmentation?
This is an example of complete bilateral sixth 2) Weakening of both medial rectus muscles.
nerve palsy occurring after severe head trauma. This The question would be, recession or Botox.
happened 18 months ago. The eyes have remained My choice would be large recession of both
like this since the accident. The eyes are stuck in medial rectus muscles plus a half tendon transfer
convergence, (actually bilateral adduction) with no shifting the lateral half of the superior and inferior
abduction. The eyes elevate and depress reasonably rectus muscles (or the full tendon) to the lateral rectus
well indicating functioning of the vertical recti and muscle. Augmentation as suggested by Foster could
obliques. The man has constant diplopia. be done at the discretion of the surgeon. Bilateral
sixth nerve palsy patients have diplopia even with
otherwise good results from surgery. Because of both
eyes being affected, a secondary deviation can occur
in any field of gaze. At best a small field of single
binocular vision can be gained. Suppression is a
comfort for these patients. The decision to do a full
tendon or a half tendon transfer would depend on the
surgeons concerns about anterior segment ischemia
occurring since only one anterior ciliary artery, that in
the lateral rectus, would remain.
339
Chapter 15
Measurements
60 pd ET primary
HAN0105
History Comment
46 year old male This man could be treated with a muscle transfer
Vision: OD 20/25, OS 20/20 of the right eye moving the lateral half of the superi-
Refraction: OD +0.75 or and inferior rectus muscles (or the full tendon) to
OS +1.00 the insertion of the right lateral rectus muscle with or
without augmentation. The right medial rectus would
This man sustained bilateral sixth nerve palsy be recessed. In the left eye, because some lateral rec-
after closed head trauma. The right eye is more tus function remains, a large recession of the medial
severely affected than the left. Trauma occurred more rectus and resection of the lateral rectus could be
than a year ago. The condition of the eyes has done. This man has a chance of having a slightly big-
remained stable for nearly a year. The right eye ger window of single binocular vision compared to
remains in the adducted position even in full dextro- a patient with bilateral total sixth nerve palsy. The
version. In contrast, the left eye is able to move out decision to do a full tendon or a half tendon transfer
at least to the midline. Testing with saccadic veloci- would depend on the surgeons concerns about anteri-
ty shows slightly brisker outward movement of the or segment ischemia occurring since only one anteri-
left eye in attempted abduction compared to the right. or ciliary artery, that in the lateral rectus, would
Generated force is nil to abduction in the right eye remain.
and a slight tug is felt in the left eye on attempted
abduction.
340
Telemedicine: distance medicine
Measurements
RT ortho right
P 60 pd ET primary
LT 100 pd ET left
HAN0055
History Comment
This man suffered a carotid-cavernous sinus fis- The chance of achieving a useful area of single
tula three years ago. He was successfully treated but binocular vision is greater in the case of unilateral
has a residual complete left sixth nerve palsy. He is palsy compared to one with bilateral involvement. In
eager to have his eyes realigned. a case like this, a question that often arises is: should
the antagonist medial rectus be injected with Botox in
the acute stage to prevent contraction? While this has
not been shown to contribute to better results for the
treatment of sixth nerve palsy in the long run, I think
that it is a treatment that should be considered if
Botox is readily available.
Surgical treatment of this case would be transfer
of the lateral half of the superior and inferior rectus
muscles (or the full tendon) to the lateral rectus mus-
cle with or without augmentation and recession of the
left medial rectus muscle. The decision to do a full
tendon or a half tendon transfer would depend on the
surgeons concern about anterior segment ischemia
occurring since only one anterior ciliary artery, that in
the lateral rectus, would remain.
341
Chapter 15
Measurements
20 pd ET primary
DRR0062
History Comment
42 year old male In a case like this it necessary to wait a sufficient
Vision: OD 20/20, OS 20/40 time to determine how much recovery will occur. In
Refraction: OD +1.00 the meantime precautions should be taken to protect
OS +1.00 the right cornea from exposure caused by the paraly-
sis of the facial muscles responsible for closing the
This man had removal of a benign brain tumor right eye and innervated by the seventh nerve. The
four months ago. He is recovering well but has had right medial rectus could have been injected with
since surgery a right esotropia with inability to abduct Botox earlier to prevent right medial rectus contrac-
the eye, and a drooping of his right face. This right ture. After eight months to a year the condition can
sixth and seventh nerve palsy is improving slowly. be considered stable and surgery can be done. The
type of surgery would depend on results of forced
ductions. If they are free, then a full tendon transfer
shifting the right vertical recti to the right lateral rec-
tus would be a good choice. If the medial rectus were
found tight on passive duction testing, then this mus-
cle would be recessed and a decision would be made
to do a full or a half tendon shift depending on con-
cerns regarding anterior segment ischemia. The right
lid lag could be helped with a partial tarsorraphy or a
more extensive facial muscle procedure by an oculo-
plastic surgeon.
342
Telemedicine: distance medicine
Measurements
100 pd ET primary
ROL0135
History Comment
44 year old male Treatment in this case would consist of:
Vision: OD 20/20, OS 20/30 1) Bilateral transfer of the vertical rectus muscles
Refraction: OD + 0.50 to the lateral rectus muscle. The question
OS + 0.50 +0.75 x 80 degrees would be, full tendon or half tendon? With or
without augmentation?
This man suffered severe head trauma one year 2) Weakening of both medial rectus muscles.
ago in an auto accident. His eyes have been crossed The question would be, recession or Botox.
since the accident. He is not able to abduct the eyes My choice would be large recession of both medial
even to the midline. He has diplopia but the images rectus muscles plus a half tendon transfer shifting the
are so far apart that he is not particularly bothered. lateral half of the superior and inferior rectus muscles
He turns his head to cross fixate because the eyes (or the full tendon) to the lateral rectus muscle.
remain in full adduction. Augmentation as suggested by Foster could be done
at the discretion of the surgeon. Bilateral sixth nerve
palsy patients have diplopia even with otherwise
good results from surgery. Because of both eyes
being affected, a secondary deviation can occur in
any field of gaze. At best a small field of single
binocular vision can be gained. Suppression is a
comfort for these patients. The decision to do a full
tendon or a half tendon transfer would depend on the
surgeons concerns about anterior segment ischemia
occurring since only one anterior ciliary artery, the
one in the lateral rectus, would remain.
343
Chapter 15
Measurements
40 pd ET
HAN0010
History Comment
2 year old girl This is an example of Class I Duane syndrome.
Vision: fixes and follows well with either eye There is a left esotropia in the primary position, lim-
Refraction: OD +2.50 ited abduction in the left eye, and narrowing of the
OS +3.00 left palpebral fissure on dextroversion. The girl
assumes left face turn and right gaze to achieve
This girl was noted by her parents to have in- aligned eyes presumably with fusion. This may be
turning of the left eye from shortly after birth. As she treated surgically with a recession of the left medial
began sitting up and walking they noted that she turns rectus muscle and possibly with a posterior fixation
her head to the left and assumes right gaze. The child suture on the right medial rectus to limit excursion in
is healthy and there is no family history of strabismus. left gaze making the eyes more nearly matched in
that gaze position. In a case like this, it is always nec-
essary to rule out a left sixth nerve palsy. The nar-
rowing of the left palpebral fissure with enophthal-
mos helps confirm Duane. This would not occur in a
sixth nerve palsy. At surgery, resistance to forced
abduction in the right eye would also be seen in
Duane, but could also be seen, probably to a lesser
degree, in sixth nerve palsy.
344
Telemedicine: distance medicine
Measurements
20 pd XT primary
ROL0065
History Comment
8 year old girl This is a class II or exotropic Duane affecting the
Vision: OD 20/20, OS 20/20 left eye. Abduction in the left eye is quite good. This
Refraction: OD +1.00 girl turns her face to the right (toward the normal eye)
OS +1.25 and assumes left gaze where she has single binocular
Stereo: fly (3000 sec) vision and where she can fuse at least the stereo fly
(3000 sec). Note in picture 3 that the inferior orbital
This girl has been noted by her parents to have a septum pushes out making the lower lid fuller. This
face turn to the right . They do not notice anything occurs because the enophthalmos caused by the co-
else about her eyes. She is otherwise healthy and has contracture of the medial and lateral recti displaces
no complaints. orbital fat. This class II Duane affecting the left eye
is treated by recession of the left lateral rectus muscle.
If the enophthalmos were more severe, both the medi-
al and lateral rectus of the involved eye could be
recessed and then the lateral rectus of the sound eye
could be recessed to treat the extra exodeviation
caused by the medial rectus recession in the affected
eye.
345
Chapter 15
Measurements
ortho
RO - CHB0011
History Comment
9 year old girl This girl has a class III or straight eye Duane
Vision: OD 20/20, OS 20/25 with a prominent upshot in adduction. Because she
Refraction: OD +0.50 has excellent stereopsis and no complaints, any surgi-
OS +0.50 cal treatment should take these two facts into consid-
Stereo: 9/9 (40 sec.) eration. It the enophthalmos and upshoot become
enough of a problem, surgery on the left eye could be
This girl is reported by her parents to have a done consisting of a recession of the left lateral rectus
smaller eye on the left and that some times it disap- with a Y split and recession of the left medial rectus.
pears. She is doing well in school and has no com- Note that the main problem with this girls appear-
plaints. ance is in far right gaze seen in picture 4. This posi-
tion is easy for her to avoid. This girl can find dou-
ble vision as can virtually all Duane patients both
before and after even successful surgery. Patients
with Duane can be made better but not perfect.
346
Telemedicine: distance medicine
Measurements
ortho primary
GUM0057
History Comment
40 year old female It has been my experience that children with
Vision: OD 20/40, OS 20/20 Duane syndrome seldom if ever complain and that
Refraction: OD +0.25 -1.25 x 180 degrees adults almost always complain. In other words,
OS +0.25 Duane syndrome becomes more symptomatic with
Stereo: fly (3000 sec.) age without necessarily any change in the eye move-
ment behavior. It is well understood that Duane can-
This woman is becoming increasingly uncom- not be cured only made some better. This is
fortable about the way she looks. She complains that achieved by normalizing head posture, reducing or
her eyes just don't look like they should. eliminating the primary position deviation, and
Specifically she states that her right eye looks small- increasing the field of single binocular vision.
er and that it does not go all the way to the right. She However these patients will always be able to find
also experiences double vision when she looks to the double vision and will continue to experience some
right. over and/or under action of selected extraocular mus-
cles. For this reason it would be unwise in my opin-
ion to offer surgery to this patient. She is probably as
well off as she can be. Surgery at best would offer no
significant improvement and at worst would create a
new problem.
347
Chapter 15
Measurements
40 pd XT - up
60 pd XT - primary
80 pd XT - down
DRD0052
History Comment
6 year old boy A large right exotropia is present in the primary
Vision: OD 20/20, OS 20/20 position. The patient deals with this by turning his
Refraction: OD +0.50 head to the left. With this posture he demonstrates
OS +0.50 stereo acuity. Abduction in the right eye is nearly
Stereo: fly (3000 sec.) normal, but adduction is limited. During levoversion
the right eye becomes enophthalmic, and it shoots
This boy has had a face turn according to his par- up or down. An A pattern is present. To treat this
ents since the time the child sat up and walked. At case surgically the following could be done:
times they see his eyes deviated outward and some- 1) Large recession of the right lateral rectus with
times the right eye looks small. This boy does not Y split
complain about his eyes. 2) Moderate recession of the right medial rectus
3) Recession of the right lateral rectus
The amounts of surgery must be determined at
the time of surgery and would depend, at least in part
on the tightness of the muscles.
348
Telemedicine: distance medicine
Measurements
25 pd XT primary
ROL0025
History Comment
13 year old girl This class II Duane is unusual because abduction
Vision: OD 20/20 OS 20/20 seems more affected than adduction in the involved
Refraction OD +0.50 OS + 0.75 eye. But the exotropia and upshoot make this more of
Stereo: 6/9 dots (80 sec.) a class II than anything else.
Surgery for this patient could consist of:
This girl has a moderate right exotropia in the 1) A moderate right lateral rectus recession with
primary position. She assumes a left face turn to keep one muscle width upshift.
her eyes in right gaze where she has excellent fusion. Since this could further limit the already slightly
There is moderate limitation of abduction in the right limited abduction in this eye. Another alternative
eye, narrowing of the right palpebral fissure on lev- would be:
oversion, enophthalmos of the left eye and both up 2) Recession of both the lateral and the medial
and down shoot of the right eye in levoversion rectus in the right eye and a recession of the
depending on whether the right eye is slightly above lateral rectus in the left eye. The right lateral
or below the midline. This girl does not complain of rectus would be shifted up one muscle width.
diplopia. Her parents are concerned about the face In addition, the patient could have:
turn. 3) Posterior fixation suture of the left medial red-
ctus to help balance alignment in right gaze
349
Chapter 15
Measurements
20 pd ET distance
8 pd XT near primary
TRK0005
History Comment
20 year old male Surgery for the enophthalmos and upshoot in
Vision: OD 20/25, OS 20/25 this patient could include:
Refraction: OD +1.00 +0.25 x 90 1) Small recession of the left lateral rectus with
OS +1.50 Y split
Stereo: fly (3000 sec.) 2) Moderate recession of the left medial rectus
This man's alignment goes from an esotropia to This could also benefit the exotropia in
an exotropia in the primary position comparing dis- downgaze
tance and near. He has severe limitation of abduction
and adduction in the left eye with enophthalmos and
mostly upshoot of the left eye in adduction. He is
most concerned with his face turn and the hyperdevi-
ation of the left eye. He also complains of frequent
diplopia when he is driving a car.
350
Telemedicine: distance medicine
Measurements
60 pd XT primary
HAN0011
History Comment
5 year old boy This boy demonstrates another striking ocular
Vision: OD 20/60, OS 20/30 motility finding that the parents did not observe.
Refraction: OD -1.00 When he looks far to the right the left eye diverges or
OS -2.00 goes to the left! This had been called perversion of
the extra ocular muscles. It is also called simulta-
This boy according to his parents has had a large neous abduction. I believe it is a very extreme exam-
exotropia since birth. He has always turned his face ple of class II Duane. In these patients the eyes are so
to the right and looked at them with the eyes in left exotropic that when co-contraction occurs in the
gaze. The child is otherwise healthy and is develop- affected eye, the mechanical advantage of the
ing normally. involved eye is so great because of the eye position in
exodeviation that the eye undergoes abduction rather
than retraction. I call this class IV Duane.
Surgical treatment of this could be:
1) Large bilateral lateral rectus recession
2) Left medial rectus resection
This breaks the rule of not resecting muscles in
cases in Duane. However, the exodeviation is so
great that I believe resection is advisable. I have
seen and treated only a few of these cases. They are
rare. I believe this is a type of Duane that deserve a
class of its own
351
Chapter 15
AL0040
352
Telemedicine: distance medicine
Measurements
Primary ortho
with head tilt
IN0047
AL0029
Measurements
25 pd ET 10 pd
right hypo primary
AL0025
A, Typical Brown with chin up head posture; B, Typical Brown; C, Brown and
esotropia - the boy does not assume a chin up head posture
Comment
These three patients demonstrate the similarities
of findings in patients with Brown syndrome. These
findings are for the most part like those of case 60 and
treatment would be similar. However note that in the
third patient there is a large angle esotropia in the pri-
mary position. This would require at least a medial
rectus recession along with the surgical treatment of
the Brown. The patient with esotropia and Brown
does not assume a chin up head posture because he
has suppression A head posture in this case is not
needed to avoid of diplopia and achieve fusion.
353
Chapter 15
Measurements
20 pd R hypo
AL0018
History Comment
9 year old boy Any time trauma around the eye causes a
Vision: OD 20/20 OS 20/20 hypotropia a blowout fracture must be suspected.
Refraction: OD plano OS plano This was not shown on the CT. I suspect that the
problem is with the right superior rectus in spite of the
This boy was stuck above the right eye with a CT findings suggesting a normal muscle. My recom-
pointed stick about one year ago. His right eye has mendations for treatment are the following:
been lower since that time. He holds his chin up and 1) At surgery confirm the presence of free forced
reports seeing double. A CT of the orbit shows the elevation of the right eye
superior rectus in place and there is no evidence of a 2) Explore the right superior rectus area and
blow out fracture. advance/repair a lacerated muscle or resect an
intact muscle
3) If passive ductions are restricted, the reason
must be found and the restrictions freed. A
decision must then be made regarding reces-
sion of the muscle associated with the restric-
tion
Trauma cases must be dealt with on an individual
basis with surgery done in response to the unique
findings of each case.
354
Telemedicine: distance medicine
Measurements
5 pd L hyper up
25 pd L hyper primary
35 pd L hyper down
AFT-kbl0007
History Comment
16 year old boy The inferior rectus is the muscle most frequently
Vision: OD 20/20 OS 20/20 involved in trauma like this. This may be due to the
Refraction: OD plano OS plano fact that the protective Bell phenomenon places the
inferior rectus in harms way as the eye rotates
This boy was struck in the left eye one year ago upward. The first attempt at repair was unsuccessful.
with a carpet hook. He was treated surgically for a The significant lower lid ptosis suggests that the mus-
lacerated left inferior rectus, but the left hypertropia, cle is there but was was not properly reattached.
limited depression of the left eye and diplopia remain. Surgery for this patient would consist of:
1) Exploration of the left inferior rectus
2) Advancement of the inferior rectus to the orig-
inal insertion. A tandem adjustable suture
could be used.
The presence of lower lid ptosis and the fact that
some depression of the left eye remains suggests that
the inferior rectus continues to act and that if it is reat-
tached, the lid will come up and the eye will go down!
355
Chapter 15
HA0004
Comment
This case may well be treated best by avoiding
surgery.
GUM0036
Comment
A trauma case like this deserves a thorough eye
examination in addition to evaluation of motility. In
this patient both were normal except for the extensive
ecchymosis and subconjunctival hemorrhage. In
cases like this the hemorrhage is said to resolve in
fourteen days with treatment and in two weeks with-
out!
356
Telemedicine: distance medicine
GUM0068
Comment
This small conjunctival laceration can be closed
with one suture of it can be allowed to heal on its
own. In a case like this be sure to rule out lateral rec-
tus damage and perforation of the globe. With an
injury like this a thorough eye examination should be
done.
DRD0066
DRD0085
Comment
These two patients are excellent examples of the
value of the Speilmann translucent occluder for
demonstrating dissociated vertical deviation in a way
suitable for photographic documentation.
357
Chapter 15
Measurements
15 pd R hypo primary
ROL0136
History Comment
16 year old girl Hypotropia in a poorly seeing eye which also
Vision: OD 20/400 OS 20/20 bobs up and down with nystagmoid movements is
Refraction: OD +3.00 OS +1.25 characteristic of the Heimann-Bielschowsky phenom-
enon. This patient also demonstrates a DVD response
This girl started with congenital esotropia and when the right eye is covered. Behavior of this sort
has undergone three surgeries. She has had no treat- can occur in a poorly seeing eye of long standing. I
ment for amblyopia. She notes that her right eye is have seen similar behavior in more than a dozen such
lower than the left and that it sometimes moves up cases. The best treatment for this patient in my expe-
and down. rience is recession of the inferior rectus of the
hypotropic right eye. In cases like this that I have
treated, there has been no exacerbation of the hyper
response of the DVD.
358
16
Strabismus case management
CASE 1 Congenital esotropia without nystagmus, CASE 18 Iatrogenic Brown syndrome, 384
363
CASE 19 Duane syndrome with esotropia (class I),
CASE 2 Congenital esotropia with nystagmus, 385
limited abduction, and face turn
(Ciancia syndrome), 365 CASE 20 Duane syndrome with limited adduction
(class II), 386
CASE 3 Nystagmus blockage syndrome, 366
CASE 21 Duane syndrome with straight eyes and
CASE 4 Residual esotropia, 367 limited abduction and adduction
(class III), 387
CASE 5 Exotropia after surgery for esotropia
(with normal or nearly normal adduc- CASE 22 Duane syndrome with simultaneous
tion), 368 abduction (class IV), 389
CASE 6 Exotropia after a slipped medial rectus CASE 23 Class I superior oblique palsy, 390
muscle, 369
CASE 24 Class II acquired superior oblique palsy,
CASE 7 Exotropia caused by a lost medial 392
rectus muscle, 370
CASE 25 Large-angle class III congenital superior
CASE 8 V pattern exotropia with overaction of oblique palsy 393
the inferior obliques, 371
CASE 26 Large class IV acquired superior oblique
CASE 9 Dissociated vertical deviation (DVD), palsy, 395
373
CASE 27 Bilateral superior oblique palsy, 397
CASE 10 A esotropia after bimedial rectus
recession, 375 CASE 28 Canine tooth syndrome: class VII
superior oblique palsy, 399
CASE 11 A exotropia after bimedial rectus
recession, 376 CASE 29 Congenital absence of the superior
oblique tendon, 401
CASE 12 Basic pattern intermittent exotropia, 377
CASE 30 Thyroid ophthalmopathy
CASE 13 Divergence excess intermittent exotropia, (Graves ophthalmology), 402
378
CASE 31 Thyroid ophthalmopathy (Graves
CASE 14 Convergence insufficiency intermittent ophthalmology) with postoperative
exotropia, 379 slippage of the recessed inferior rectus,
403
CASE 15 Persistent diplopia after surgery for
intermittent exotropia, 380 CASE 32 Thyroid ophthalmopathy (Graves
ophthalmology) involving multiple
CASE 16 Congenital Brown syndrome, 381 muscles, 405
CASE 17 Acquired Brown syndrome, 383 CASE 33 Unilateral sixth nerve palsy, 406
359
CASE 34 Bilateral sixth nerve palsy, 408 CASE 52 Ocular myasthenia, 431
CASE 35 Bilateral sixth nerve palsy with CASE 53 Absence or the medial rectus muscle,
persistent diplopia after successful 432
treatment, 409
CASE 54 Traumatic disinsertion of the inferior
CASE 36 Right sixth nerve palsy from rectus muscle, 433
intracranial aneurysm, 411
CASE 55 Diplopia after cataract extraction from
CASE 37 Acquired third nerve palsy, 412 left inferior rectus restriction, 435
CASE 38 Traumatic third nerve palsy with mis- CASE 56 Diplopia after repair of retinal detach-
direction after successful horizontal ment, 437
alignment, 414
CASE 57 Diplopia after repair of retinal detach-
CASE 39 Congenital third nerve palsy, 416 ment, 438
CASE 40 Severe bilateral congenital third nerve CASE 58 V pattern esotropia with overaction of
palsy, 417 the inferior oblique muscles, 439
CASE 41 Sensory exotropia, 418 CASE 59 A exotropia, bilateral overaction of the
superior obliques, dissociated vertical
CASE 42 Residual sensory esotropia, 419 deviation, 441
CASE 43 Dissociated vertical deviation with true CASE 60 Parinauds paralysis of elevation, 443
hypotropia (falling eye), 420
CASE 61 Null point nystagmus, 444
CASE 44 Double elevator palsy, 422
CASE 62 Congenital nystagmus with decreased
CASE 45 Blowout fracture of the orbit, 423 vision, 446
CASE 46 Acute blowout fracture of the orbit, 424 CASE 63 Nystagmus after brain stem stroke, 449
CASE 47 Congenital fibrosis syndrome, 425 CASE 64 Superior oblique myokymia, 450
CASE 48 Mbius syndrome, 426 CASE 65 Typical refractive esotropia, 451
CASE 49 Skew deviation with symptomatic CASE 66 Refractive/ accommodative esotropia
diplopia, 427 (high AC/A), 452
CASE 50 Acquired esotropia, 428 CASE 67 Refractive esotropia with dissociated
CASE 51 Chronic progressive external ophthalmo- vertical deviation, 453
plegia, 430
360
Strabismus case management
Case presentations
The layout of the patient presentations in this the large heading congenital esotropia syndrome.
section includes the following: The latter includes all patients born with the potential
1. A clinical photograph of the patient empha- for motor fusion and comprises the remainder of stra-
sizing the most informative character- bismus in its many and varied forms.
istic(s). The algorithm of etiology recognizes five princi-
2. A brief clinical history highlighting perti- pal causes for strabismus or misalignment of the eyes:
nent facts about this patient. These histori- 1. Congenital absence of motor fusion, leading
cal items will be typical of the class of to congenital esotropia and its sequelae
patient presented. 2. Congenital or acquired mechanical strabis-
3. Pertinent clinical measurements including mus
those motor and sensory findings important 3. Congenital or acquired supranuclear,
in diagnosis and treatment planning. nuclear, and fascicular neural strabismus
Although the complete motility examination including intermittent exotropia
as described previously (see chapter 4) has 4. Congenital or acquired sensory deficit lead-
been completed for all patients up to the ing to strabismus
level of the patients ability to cooperate only 5. Refractive-accommodative esotropia
selected findings will be described. In the first category with absence of the motor
4. Diagnosis fusion mechanism in the occipital cortex, the eyes are
5. Nonsurgical treatment not guided gently and inexorably to alignment during
6. Surgical treatment the formative months of life. In the absence of this
7. Comments central guide, called motor fusion, the peripheral
The management of strabismus starts with recog- motor elements (extraocular muscles guided by
nition of the entity; a diagnosis must be made. After supranuclear vergence mechanisms and activated
thinking about how this is actually done, a fairly through motor nerves) tend to go their own way.
unlikely solution occurred to me. The diagnosis of Since the most exuberant infantile oculomotor
strabismus in most cases is made by first recognizing response is convergence, esotropia is the result of a
the qualitative findings and then assigning a diagnos- lack of central motor fusion. Later, this lack of motor
tic label. Third nerve palsy looks like third nerve fusion control has its effect on some subtle and not so
palsy because the eye is down and out with ptosis and subtle brainstem oculomotor control functions and
frequently a large pupil. Congenital esotropia looks produces dissociated vertical deviation (DVD),
like congenital esotropia because the eyes are crossed oblique dysfunction, asymmetric optokinetic nystag-
in an infant who can abduct either eye. The diagno- mus (OKN), torticollis, and latent nystagmus.
sis of strabismus is made by simply knowing and rec- Now for the rest of strabismus! It may seem to
ognizing this condition in a patient. Although a thor- be painting with too broad a brush to assign the
ough understanding of mechanisms of strabismus and remainder of strabismus to a single branch, those born
a thorough knowledge of the diagnostic routine are with motor fusion who develop strabismus on the
essential, the diagnosis is made more on the basis of basis of mechanical or neural factors. I hope I can
observation than by the deductive process. Each stra- convince you that this is logical. While all of these
bismus has its gestalt, and awareness of this is the patients in the second branch of the classification
foundation of strabismus diagnosis. Once the diag- have in common the capacity for motor fusion, some
nosis is made, it is necessary to work more or less retain normal sensory fusion at least part of the time,
backward from the diagnosis by determining size or some lose it (acquired strabismus with suppression),
amount (quantification), the sensory status, and final- and others may never have been able to realize the
ly, the etiology of the strabismus. The ability to carry potential (Mobius syndrome, congenital third nerve
out this process beforehand is essential to the ultimate palsy). Depending on time of onset of the strabismus,
effective treatment of strabismus. inability (ability) of the eyes to attain alignment with
A key to the understanding of strabismus starts vergence response or by assumption of an appropriate
with etiology. The concept can be demonstrated in an head posture, and providing there is equal sensory
algorithm (p.24). The first branching of this algo- input, fusion potential can be salvaged. On the other
rithm separates all of strabismus into those patients hand, in the absence of these factors it can be lost. In
with inborn errors in motor fusion mechanism, the a similar way, sensory fusion (stereopsis) may be
basic cause of congenital esotropia, and those born retained if favorable factors prevail or it may be lost.
with motor fusion potential. The former category Diplopia from defective motor fusion may be either
extends linearly to include the broad spectrum of ocu- constant or intermittent. Finally, suppression--either
lar motility disturbances, which can be placed under intermittent and reversible, as in intermittent
361
Chapter 16
362
Strabismus case management
Clinical picture
A B
A 6 month-old boy with 40 prism diopters of congenital esotropia A, before and B, 1 day after bimedial rectus
recession to 9.5 mm from the limbus with a limbal approach.
History
This patient with congenital esotropia was prism diopters esotropia), with a range of 10 to 90
brought in for examination at 6 months of age; simi- prism diopters. No A or V pattern is observed in
lar infants are usually seen initially between 4 and 12 the straightforward case, although these findings and
months of age. Parents often state that the eyes are DVD may be seen at the initial examination of the
crossed or that they do not track together. The older child with congenital esotropia. Throughout the
infants general health, especially neurologic status, is examination one should closely observe for both
normal. Older children, up to 2 or 3 years old or manifest and latent nystagmus, including fine mani-
more, may be examined for the first time with a sim- fest rotary movements. The patient shown here did
ilar picture and be diagnosed as probably congenital not have nystagmus. This is a very important finding,
esotropia based on the history. In either case, parents since in my experience patients without nystagmus
tend to report that the eyes have been crossed since have better surgical results. Refraction is done 40
birth. However, after specific questioning about the minutes after 1 drop of Cyclogyl has been instilled in
timing and duration of the crossing, they may admit each eye (1/2% in infants under 1 year and 1% in chil-
that the esotropia was intermittent at first and con- dren over 1 year). Retinoscopy findings are typically
stant later. One or both parents, a sibling, or another between plano and less than +3.00 diopters. This
relative may have strabismus, but the family history infant had retinoscopy of +1.00 diopter in each eye.
may be negative for strabismus. The examination is completed with evaluation of the
anterior segment, media, and retina (posterior pole),
Examination including evaluation of the optic nerve and macula.
An infant must be approached gently, with quiet
reassuring movements, in order to maintain some
Diagnosis
semblance of cooperation. The child is observed for Congenital esotropia without nystagmus,* 40
fixation and following behavior of each eye, using an prism diopters, alternating.
interesting object and employing a nonthreatening
cover test. Lateral versions should be observed to Treatment
confirm full abduction. If full abduction is not If amblyopia is diagnosed by noting fixation
accomplished while the infant is following an inter- preference, occlusion therapy is started following one
esting object, the dolls head or oculocephalic of these two techniques:
maneuver should be done to rule out limited abduc- Patch preferred eye all waking hours. Check
tion as the cause of the esotropia. Prism and cover in 1 week for infants under 1 year of age, in 2
testing is difficult in the infant. The deviation can be weeks for children over 1 year of age, and in
measured with sufficient accuracy using the Krimsky suitably short intervals in any older child to
prism test or the Hirschberg light reflex test with the accurately monitor the fixation behavior while
infant looking across the room. The average devia- avoiding occlusion amblyopia. Continue
tion is 30 to 45 prism diopters (this infant had 40 occlusion until alternation is achieved or no
*This type of congenital esotropia can demonstrate latent nystagmus with DVD later in its course, after surgical treatment
363
Chapter 16
improvement is noted after 3 months of patch- esotropia. I estimate that 10% of patients will need a
ing with compliance. Before discontinuing second surgery, usually for residual esotropia, in the
attempts at patching, a thorough reassessment first year. Seven of 10 will require a second or third
of the physical status of the eye should be car- surgery before their teens. Parents are told that a
ried out. child treated surgically for congenital esotropia has a
Patch preferred eye 3 or 4 days, then patch the likelihood of needing additional surgical procedures
amblyopic eye 1 day in continuing cycles (one for an early over- or undercorrection and/or for new
eye is always patched), and follow the routine strabismus occurring after a period of alignment.
described above. This technique provides a These additional surgical procedures are required for
safer program to avoid occlusion amblyopia, DVD, oblique overaction with V or A pattern,
especially in cases where close follow-up is not residual esotropia, and secondary exotropia. It must
possible. be emphasized that the patient with congenital
If +3.00 D hyperopia or greater is found at cyclo- esotropia has an abnormal central nervous system
plegic refraction in a patient similar to the one shown subserving motor fusion. Surgery on the exrtraocular
here, spectacles are usually prescribed. If the eyes are muscles combined with amblyopia treatment when
aligned with spectacle correction, refractive esotropia indicated improves alignment and maximizes sensory
is confirmed and the treatment is continued glasses function, but this does not eliminate the original and
wear. If the eyes are not aligned with the glasses, underlying neurologic problem. The best result
congenital esotropia is confirmed. In my experience obtainable from treatment for congenital esotropia is
this is the usual case in infants under 1 year. Since subnormal binocular vision.
low plus correction of +3.00 or +4.00 diopters Patients treated surgically for congenital
rarely results in alignment, loaner glasses may be pro- esotropia require close follow-up until their teen
vided to cut down on the expense for parents. years. It is especially important to monitor amblyopia
in the first few years after the eyes are aligned
Surgery because it is more difficult to assess fixation behavior
An infant 4 months of age or older with and therefore amblyopia in the preverbal child after
esotropia without amblyopia and without a refractive successful surgery. Other sequelae such as DVD,
component is a candidate for eye muscle surgery. My oblique dysfunction, and exotropia must also be
choice of surgical treatment for congenital esotropia watched for. I tell families of infants treated surgi-
without nystagmus is bimedial rectus recession of cally for congenital esotropia that they need an oph-
between 8.5 and 11.5 mm measuring from the limbus, thalmologist for a friend at least until the child
with the amount of surgery depending on the age and receives a drivers licence (that is, until the mid-
the angle. Deviations are divided into small, medium, teens).
and large and bimedial rectus recessions are likewise For any type of congenital esotropia, some stra-
divided. bismologists use injection of Botox into one medial
rectus muscle in doses up to 5 units. This has been
Bimedial rectus recession reported to produce alignment of 10 dipoters or less
Deviation (mm) <1 yr >1 yr >5 yr esotropia in just over 60% of patients with an average
Small 20-30 diopters 8.5 9.0 9.0
of 1.7 injections. Newer studies by Campos report
Medium 30-45 diopters 9.5 10.0 10.5
Large 45+ diopters 10.5 11.0 11.5 88% alignment in congenital esotropia patients after
10.0 mm is the maximum between 4 and 6 months injection of 5 units of Botox in each medial rectus
under direct observation with the patient under gener-
In practice, there is a significant overlap in the pre- al anesthesia. This compares to similar alignment in
ceding table. The inconsistencies can only be just over 80% of patients after bimedial rectus reces-
described as the art of strabismus surgery. In my sion. Some surgeons prefer three or four horizontal
opinion, any surgeon who adheres doggedly to a for- rectus muscle surgery for larger angle esotropia,
mula will produce inferior results. Minor adjust- adding one lateral rectus resection for esotropia
ments should be made based on subtleties of each greater than 70 diopters. I do bimedial rectus reces-
patients strabismus. In addition to bimedial rectus sion as the first surgical procedure for all patients
recession, conjunctival recession after limbal incision with congenital esotropia. We produce hard to
may be done in patients with an angle over 70 prism explain undercorrection with some small angles and
diopters and in cases where passive abduction is overcorrection with some larger preoperative
found to be restricted at the time of surgery. esotropia in patterns that suggest that factors other
than just the angle of deviation predict the response to
Comment surgery.
With appropriate surgery 80% to 85% of eyes
will be aligned with less than 10 prism diopters of
residual strabismus, which is in most cases an
364
Strabismus case management
History Treatment
This 10-month-old boy presented with a typical Amblyopia and hyperopia (refractive compo-
congenital esotropia history: the eye crossing from nent) if present are treated in the usual manner.
birth or shortly after in an otherwise normal infant.
This patient differs in that his parents say he appears Surgery
to have both eyes crossed and usually turns his head. Bimedial rectus recession is performed accord-
ing to the angle. A slightly larger bimedial rectus
Examination recession is indicated compared to congenital
Abduction is apparently limited, but with effort esotropia without nystagmus. As a guideline, I add
it is full with nystagmus increasing in abduction. 0.5 mm to 1.0 mm to each medial rectus recession,
Nystagmus (manifest latent nystagmus) is present in according to age. The maximum for this child is 10.5
the primary position with the slow phase toward the mm from the limbus. Some surgeons would add a lat-
nonfixing eye. eral rectus recession in one eye.
The strabismus angle is measured with the
Krimsky prism test or the Hirschberg light reflex test. Comment
In this patient 60 prism diopters of esotropia is meas- Congenital esotropia with nystagmus is more
ured. Prism and cover testing is more difficult to per- likely to result in postoperative undercorrection when
form than in congenital esotropia without nystagmus. a similar amount of surgery is done compared to con-
The angle is usually larger than congenital esotropia genital esotropia without nystagmus. This means that
without nystagmus by 10 prism diopters or more. close follow-up is needed. Repeat surgery for a sig-
The remainder of the examination is normal. nificant residual angle is needed about twice as often
Retinoscopy after 1/2% Cyclogyl is OD + 1.50, OS as in congenital esotropia without nystagmus.
+1.50. The ultimate in manifest latent nystagmus
occurs in infants who have no vision in one eye or
Diagnosis have undergone enucleation of one eye. These
Congenital esotropia with nystagmus (Ciancia patients may develop pronounced manifest latent nys-
syndrome). tagmus with the slow phase toward the non-seeing
eye and face turn toward the seeing eye. Improved
head posture results after recession of the medial rec-
tus and resection of the lateral rectus of the sound eye.
365
Chapter 16
Clinical picture
366
Strabismus case management
Clinical picture
A B
History
This 14-month-old boy had a bimedial reces- 11.0 mm limit for initial surgery . If less than 2.0 mm
sion to 9.5 mm from the limbus (equivalent 4.5 mm of re-recession can be done; that is, if more than 9.5
from the insertion) at 4 months of age for 35 prism mm bimedial rectus recession or equivalent had been
diopters of esotropia. The eyes were never aligned done at the initial surgery; less effect would be gained
postoperatively and now 25 prism diopters of residual from re-recession and I would consider lateral rectus
esotropia remains. resection. In a young child with residual esotropia, I
would rarely elect to operate on just one muscle.
Examination Another choice for surgery would be re-recession of
A stable angle of 25 prism diopters esotropia is one medial rectus and resection of the lateral rectus of
measured with the Krimsky test. Ductions are full. the same eye.
No oblique dysfunction or vertical incomitance is Repeat surgery for residual esotropia is indicat-
seen. No nystagmus is noted. ed for the same reason that surgery is done originally.
An acceptable residual angle is 10 prism diopters;
Diagnosis greater than this can be reason for reoperation.
Residual esotropia after bimedial rectus However, some residual angles greater than 10
recession. diopters are not noticeable. If that is the case, I do not
think that a second surgery is necessary. After sur-
Treatment/Surgery gery this type of undercorrected patient may be noted
to have manifest nystagmus (manifest latent nystag-
Re-recession of the medial recti to 11.5 mm.
mus) and what I call ocular instability with small
Comment amplitude horizontal, vertical, and rotary nystagmus
not seen before the initial surgery. Surgical treatment
The choice of surgery depends in large part on for residual esotropia is based primarily on how the
what was done at the first surgery. If the bimedial patient looks. In contrast to young patients, some
rectus recession was less than maximum, the medial teenagers and adults with residual esotropia can ben-
recti may be re-recessed to a maximum of 11.5 mm. efit from a single medial rectus weakening procedure
Since this case was undercorrected, a slightly larger for an angle of 15 prism diopters.
recession was done in this 1-year-old, exceeding the
367
Chapter 16
Clinical picture
History Treatment/Surgery
This 22-year-old man had bimedial rectus Advancement and resection of both medial
recession to 10.0mm from the limbus (5.0 mm equiv- recti, including 4.0 mm of advancement and 2.0 mm
alent measured from the insertion) at 3 years of age. of resection.
His eyes were aligned for 3 years then they gradually
began to drift out. For the past several years he has Comment
had a lazy eye, according to his parents and friends. The most important physical finding is the
nearly full adduction, that suggests the medial recti
Examination have not slipped. In this type of patient, some sur-
Visual acuity is 20/20 in each eye without cor- geons would prefer to do a moderate bilateral lateral
rection. The exotropia measures 35 prism diopters rectus recession. In my hands recessing both lateral
distance and near with prism and cover testing. recti 5.0 to 6.0 mm would be appropriate. Another
Ductions are essentially full in either eye, except for alternative surgery is resection and advancement of
a trace of reduced adduction bilaterally. No vertical the medial rectus and recession of the lateral rectus in
incomitance, oblique dysfunction, or nystagmus is the non-preferred eye.
seen. This patient usually fixed with the left eye but
would take up and hold fixation with the right eye
when asked.
Diagnosis
Secondary exotropia.
368
Strabismus case management
Clinical picture
A B
Limited adduction, more pronounced in the left eye in a 14-year-old boy with slipped medial recti after bime-
dial rectus recession done at age 1 year for congenital esotropia. A, dextroversion; B, levoversion
History
This 14-year-old boy had bimedial rectus reces- free, but this must always be confirmed at surgery.
sion for congenital esotropia at age 1 year. The eyes When exploring these medial recti, their intended
turned out a few months after surgery and he has had point of reinsertion, if known, should be inspected
a gradually increasing exotropia for the past 10 years. meticulously. If the medial rectus is where it belongs
He now comments that his friends notice his eye at the intended or at least at a reasonable recessed
being out. This has been a concern to both the patient position, muscle tissue should be seen at this point. In
and his parents for the past several years. the case of a slipped muscle, muscle tissue will not be
seen here. Instead an empty muscle capsule will
Examination firmly adhere to the globe. With careful dissection
An exotropia of 45 prism diopters is measured this can be followed posteriorly to the muscle tissue
in the distance, and this increases to 55 prism diopters which is often found excessively recessed to a point
at near in the primary position. Visual acuity is 20/20 at or posterior to the equator. At surgery this muscle
in the right eye and 20/40 in the left with the visual must be engaged on a muscle hook, secured with a
reduction due to slight amblyopia. The salient feature suture, and brought forward to a point at or near the
in this case is reduced adduction in both eyes. Even original muscle insertion. In most cases it is difficult
with maximum attempts at adduction, a prominent to bring the muscle up to a point 5.5 mm from the
rim of sclera shows medially, indicating that the limbus (the usual original insertion.) Instead, the
medial recti are underacting. muscle is reinserted 6 to 7 mm from the limbus, hav-
ing been advanced usually 5.0 mm or more. To
Diagnosis achieve sufficient effect, I usually include a resection
Secondary exotropia after surgery for esotropia, of the muscle with the advancement. For example, I
and in addition a suspicion that the medial recti have would do a 5.0 mm advancement and a 3.0 mm resec-
slipped backward from their point of reinsertion. tion in this case. The final placement of the muscle
on the sclera is a matter of intraoperative judgement.
Treatment/Surgery Slipped medial recti may be unilateral as well as
bilateral. In the case of a unilateral slipped medial
Advancement and resection of both medial recti
rectus, the exodeviation will be incommitant and
based on the findings at surgery.
adduction will be deficient only on the side of the
Comment slipped muscle. Slipped muscles happen, in my opin-
ion, because the surgeon places the suture too close to
This type of reoperation cannot be subject to a the rectus muscle insertion before disinsertion. With
cookbook type of answer. The type and amount of a slipped muscle, the suture is more likely to slip out
surgery required depend on the findings at surgery. In of the muscle than it is to break loose from sclera.
cases such as this the passive ductions are usually
369
Chapter 16
370
Strabismus case management
Clinical picture
A B
D E
V pattern exotropia with bilateral overaction of the inferior obliques. A, up right gaze;
B, up left gaze; C, primary position; D, upgaze; E, downgaze
371
Chapter 16
History Treatment/Surgery
This 10-year-old girl had bimedial rectus reces- Bilateral inferior oblique weakening. My pre-
sion for congenital esotropia at 1 year of age. Both ferred technique for this is bilateral inferior oblique
medial recti were recessed 10.0 mm from the limbus myectomy.
for 45 prism diopters of esotropia. She did well for
several years but now the family notices that one of Comment
her eyes goes out of sight at times. They could not Overaction of the inferior obliques occurs fre-
be any more specific or explain precisely when or quently after bimedial rectus recession, occasionally
how they notice this. in congenital esotropia that has not had surgery and
also as an isolated primary finding without other stra-
Examination bismus. It is certainly legitimate to ask the question,
Visual acuity is 20/30 in each eye while wear- Does the inferior oblique truly overact? In my
ing a low hyperopic-astigmatic correction. The prin- opinion, the inferior oblique probably does overact
cipal positive finding is marked elevation of each eye but only insofar as its relationships to other structures
in adduction (strabismus sursoadductorius) with over- around the eye allow it. For example, after a bimedi-
action of the inferior obliques. In addition, a V pat- al rectus recession, the inferior oblique overacts by
tern is present with 30 prism diopters of esotropia in assuming more presence as an abductor as a result of
downgaze. Cover-uncover testing of each eye in the the altered muscle insertion relationship caused by
primary position demonstrates only the slightest trace retroplacement of the medial recti. This, I believe, is
of DVD . While the patient fixates with either eye in the reason for the exotropia in upgaze producing the
adduction, the abducted fellow eye does not become V pattern. Capo and Guyton have shown this con-
hyperdeviated under cover (as it would if DVD were vincingly. On the other hand, primary overaction of
a major factor) but instead is hypodeviated. There is the inferior obliques in cases without prior bimedial
minimal apparent underaction of the superior oblique rectus recession is, in my opinion, due to undercheck-
and ductions are otherwise normal, as is the remain- ing by the superior oblique tendon as occurs with
der of the eye examination. congenital superior oblique palsy caused by an anom-
alous loose tendon. Other instances of presumed
Diagnosis inferior oblique overaction are due at least in part to
V pattern exotropia with bilateral overaction DVD. In cases where overaction of the inferior
of the inferior obliques after bimedial rectus recession obliques is associated with DVD and a V pattern,
for congenital esotropia. inferior oblique anterior transposition is the proce-
dure of choice.
372
Strabismus case management
Clinical picture
History Diagnosis
This 5-year-old boy had bimedial rectus reces- Dissociated vertical deviation, left eye greater
sion for congenital esotropia at age 18 months. His than right eye, manifest left eye.
eyes remained aligned for several years, but recently
his mother has noticed that the left eye goes way Treatment
up, especially when the boy is tired or inattentive. In cases where DVD is asymmetrical and is
His mother estimates the left eye is deviated upward never or seldom present with a given eye fixing, fix-
more than 50% of the time. ation with this eye is encouraged and no further treat-
ment may be needed. However, if the DVD is mani-
Examination fest sufficiently often to cause distress to the patient,
Visual acuity is 20/20 in the right eye and 20/40 then surgical treatment is indicated.
in the left with best correction. A mild amblyopia is
present in the left eye. On casual observation the eyes Surgery
look aligned, but at other times the left eye is up, Asymmetrical large recession of the superior
resulting in approximately 15 prism diopters of recti. The left superior rectus is recessed 7.0 mm and
hyperdeviation. When the right eye is occluded it is the right superior rectus is recessed 5.0 mm.
approximately 5 prism diopters hyperdeviated. With
the cover removed the eye moves briskly down Comment
toward the primary position with incycloduction. Because of the peculiar manifestation of DVD
When the cover is placed over the eye again the right as an intermittent vertical vergence occurring in
eye moves slowly upward with excycloduction. A patients with imperfect fusion, the surgical treatment
similar but larger hyperdeviation with similar cyclo- of DVD cannot be expected to be specific, clear cut,
ductions occurs when the left eye is occluded. When or universally effective. On the contrary, surgical
the occluder is removed the left eye moves slowly to treatment is only moderately successful and is defi-
the primary position with incycloduction. Latent nys- nitely not the subject of widespread agreement among
tagmus of very low amplitude can be detected in the strabismologists. Some surgical options include:
fixing eye when either eye is occluded. No A or V large superior rectus recession, maximal hang
pattern is observed, and none of the oblique muscles back superior rectus recession, superior rectus poste-
overacts. rior fixation suture with or without recession, inferior
373
Chapter 16
rectus resection, and inferior oblique anterior transpo- for DVD is indicated only if a hyperdeviation is man-
sition. Superior rectus recession is the most com- ifest sufficiently often and the deviation large enough
monly employed procedure for most patients who to compromise appearance.
have DVD requiring surgery. I rarely do this surgery DVD is, in my opinion, a nonspecific manifes-
unilaterally. For persistent DVD after superior rectus tation of imperfect binocularity. It occurs most often
recession, I do inferior rectus resection. If a V pat- in the most common manifestation of imperfect
tern and inferior oblique overaction are present with binocularity, congenital esotropia. However, DVD
DVD, I do bilateral inferior oblique anterior transpo- can accompany any type of strabismus. It tends to
sition as the first surgical procedure. It must be made develop in longer standing cases and those with more
perfectly clear that the mere presence of DVD is not profound defects in binocularity. In other cases DVD
reason for surgery. More than half of all congenital can occur in patients with gross stereopsis. DVD is to
esotropia patients have some DVD after surgery, strabismus as hyperpyrexia (fever) is to infection.
including even those with the best results. Surgery
A B
A, A manifest left hypertropia. Cover/uncover testing shows a typical, unequal (OD<OS) DVD. B, When the eyes
are uncovered, they are aligned.
This 10-year-old girl manifests a 20 prism diopter left hyperdeviation shown immediately after a cover was removed.
The girl and her parents see this only rarely, such as when the child is ill with a fever. They never see the right eye
up. However, a slight right DVD can be elicited with the cover test. In this case, no surgery is indicated.
374
Strabismus case management
Clinical picture
A B C
A esotropia
A 25 prism diopters esotropia in primary position
B 35 prism diopters esotropia in upgaze
C Eyes are aligned in downgaze
History Comment
A 10-year-old boy gradually developed a lazy An A pattern vertical incomitance may occur
eye according to his mother. The boy had bimedial with or without apparent superior oblique muscle
rectus recession to 9.5 mm from the limbus for 35 overaction. On the other hand, V pattern is almost
prism diopters of congenital esotropia at age 20 always associated with overaction of the inferior
months. The deviation is especially bad when he obliques. A pattern without superior oblique over-
looks up. The boy assumes a chin-up position and action occurs in some cases when the medial recti
always seems to be looking down his nose at people, have been recessed. The pattern probably is due to
according to his mother. altered mechanics, as is the case in my opinion with
most apparent oblique muscle overaction causing
Examination greater exodeviation (less esodeviation) in upgaze or
Visual acuity is 20/30 in each eye. The eyes are downgaze. The principle of vertical displacement of
35 prism diopters esotropic in upgaze and are aligned the horizontal recti can be applied in any vertical
in 30 degrees of downgaze at distance measurement. incomitance occurring without oblique dysfunction.
In the primary position, 25 prism diopters of esotropia The medial recti are moved to the closed end and
is measured. There is no overaction of the superior the lateral recti are moved to the open end. The
obliques and no underaction of the inferior obliques. usual amount of vertical shift is one-half to one
The wings seem to stand up on the stereo fly test muscle width.
(gross stereopsis - 3000 sec) with the chin up and eyes
in downgaze.
Diagnosis
A pattern esotropia without oblique muscle
overaction or underaction
Treatment/Surgery
Re-recess both medial recti 2 mm with one
half to three quarters muscle width upshift.
375
Chapter 16
Clinical picture
A B C
History Treatment/Surgery
This 4-year-old girl had recession of both medi- Bilateral superior oblique weakening (tenecto-
al recti to 9.5 mm from the limbus at age 11 months. my or recession).
Gradually since that time she has been noted by her
parents to be wall eyed when she looks down to eat Comment
or to look at books. Her parents say she holds her When instead of an esodeviation in upgaze, as
chin down a lot and tends to look up. occurs in the preceding case, an exodeviation in
downgaze occurs, causing an A pattern, a different
Examination surgical approach is indicated. My choice, in this
Visual acuity is 20/40 in each eye with linear E case, would be bilateral weakening of the superior
vision testing. Refraction after cycloplegia is OD obliques if the eyes were aligned or nearly so, as
+1.75 and OS +1.25. The eyes are aligned in the pri- shown in the case above. If, on the other hand, an
mary position. Five prism diopters of esotropia is exodeviation greater than 10 or 15 prism diopters is
measured in upgaze and 60 prism diopters of present in the primary position, bilateral lateral rectus
exotropia is measured in downgaze. The superior recession of a small amount (3 to 4 mm) is combined
obliques are 2+ overacting. Stereo acuity is nil. The with a one-half to one muscle width downshift.
remainder of the eye examination is unremarkable. In this case, even though the superior obliques
are overacting because of the altered medial rectus
Diagnosis insertion, they are weakened. This will balance the
A exotropia after bimedial rectus recession muscle forces as they are so as to achieve alignment.
with overaction of the superior oblique muscles.
376
Strabismus case management
Clinical picture
A B
History
This 9-year-old girl was brought in by her par- recession. A recession procedure is tissue sparing and
ents, who report that their daughters eye had wan- causes less redness and tissue heaping in the anterior
dered out occasionally from the time she started part of the eye.
walking. They now see the left eye out 30% to 50% This child has had a long history of intermittent
of the time. exotropia. It is likely that the deviation had been
intermittent at distance but it gradually decompensat-
Examination ed to a nearly constant deviation. If this child had
Visual acuity is OD 20/30-1 and OS 20/25-1. been seen at age 3 instead of 9 years, a period of
Cycloplegic refraction is OD + 1.50 and OS + 1.00. observation would have been appropriate before
She fuses 6/9 stereo dots (80 seconds). The prism scheduling surgery. During the observation, parents
and cover test shows exotropia of 30 prism diopters are instructed to chart their childs deviation. This
in the distance and 25 prism diopters intermittent activity serves the dual purpose of marking the
exotropia at near. Near point of convergence is to the behavior (and progress) of the deviation and ensuring
bridge of the nose (<2 cm). No oblique over-or that the parents understand better the aims of surgery.
underaction or A or V pattern is noted. The child If in a case such as this an A or V pattern is pres-
has no symptoms, but on direct questioning her par- ent, suitable oblique muscle weakening could be car-
ents say she always closes the left eye when out- ried out or, in the absence of oblique overaction, the
doors in bright sunlight. The remainder of the eye rectus muscle insertion could be shifted upward or
examination is normal. downward according to the pattern.
Diagnosis
A B
Basic pattern intermittent exotropia.
Treatment/Surgery
Bilateral lateral rectus recession 6.0 mm.
Comment
This patient presents a typical clinical picture
of a child with basic intermittent exotropia. This may A A 6-year-old girl is shown orthotropic at near.
be treated with bilateral lateral rectus recessions or B She is exotropic (measuring 30 prism diopters) 90% of
with a recession of the lateral rectus and a resection the time according to her parents. This moderate angle
intermittent exotropia will be adequately treated with a 6.0
of the medial rectus. I prefer to avoid resecting a mm bilateral lateral rectus recession. The percent of time
muscle if a successful result can be obtained with a exodeviated does not influence the amount of surgery.
377
Chapter 16
Clinical picture
A B
History Treatment/Surgery
Over the past 2 years, this 4-year-old girl has Bilateral lateral rectus recessing 7.0 mm.
been noted by her family to have an eye that wanders
out when she is tired and when she is looking in the Comment
distance. Also, she closes her left eye almost con- This girl has a fairly classic intermittent
stantly in bright sunlight. The child has been other- exotropia, which is classified as a divergence excess
wise healthy and is doing well in preschool. intermittent exotropia because the distance deviation
is persistently larger than the near. If, after occlusion
Examination of one eye for 1 hour cover testing at near carried out
Visual acuity with correction is 20/25 in each without allowing the patient to become binocular had
eye. Retinoscopy after 1% Cyclogyl is OD +.50, OS resulted in a near deviation increasing to become
+.75. This patient fused 7/9 stereo dots (60 seconds), equal or nearly equal to the distance deviation, this
and her eyes were aligned throughout the early part of could be called a pseudo divergence excess intermit-
the examination. Cover testing revealed 40 prism tent exotropia. If the distance and near deviation had
diopters of intermittent exotropia at distance. been equal from the outset, basic exotropia would be
Recovery is fairly brisk, but the left eye does remain the diagnosis. Most patients with intermittent
exodeviated through a blink and remains exodeviated exotropia do well with surgery. However, patients
until the patient changes fixation, usually to near, or with divergence excess intermittent exotropia may
her attention is called to the fact that the eye is out. have esotropia at near postoperatively, producing
She experiences no diplopia during this manifest bothersome diplopia and requiring base-out prism.
phase. At near, prism and cover test measures 15 Such treatment may be prolonged for a few weeks or
prism diopters of intermittent exotropia. Near point months. In a few cases it has been necessary to recess
of convergence is to the nose. The remainder of the one or both medial recti in older symptomatic
eye examination is completely normal. After wearing patients. This in turn could cause a return of the dis-
a patch over the left eye for 1 hour, near cover testing tance exodeviation. This response is rare. The
was repeated without allowing any binocular experi- amount of surgery is dependent on the angle of devi-
ence, and the near deviation remained 15 prism ation. A smaller angle of exodeviation requires a
diopters intermittent exotropia. smaller amount of surgery and vice versa, but the tim-
ing of intermittent exotropia surgery is done on the
Diagnosis basis of how often the eye is deviated, not by how far
Divergence excess intermittent exotropia. the eye is out.
378
Strabismus case management
Comment
Convergence insufficiency intermittent
exotropia usually occurs in adulthood but may be
seen in children. Symptoms are usually as noted
here; that is, trouble concentrating for a prolonged
period of time on near objects with or without diplop-
ia. The near point of convergence is routinely remote.
Often, as in this case, Stereo acuity is excellent.
However, this excellent potential for binocular vision
cannot be sustained comfortably for long periods.
Some patients with this type of problem are helped by
near point of convergence, pencil push up training.
This condition may be the one best treated with
orthoptic exercises. However, many patients are
History unable to sustain comfortable near vision even with
A 35-year-old woman was seen initially with a orthoptic exercises, and surgery is needed in those
complaint that her left eye jumped up and down cases. Surgery may be a recess/resect procedure or a
and that both eyes turned out. Mild ptosis of the left bimedial rectus resection. These patients, especially
upper lid was an incidental finding. This had been those having bimedial rectus resection, often have
present for many years, according to the patient, who significant overcorrection in the early postoperative
also stated that she saw double most of the time at course. This requires temporary treatment with base-
near. She particularly had trouble with reading, caus- out Fresnel prism and of course patience and time.
ing her great difficulty in her job as a secretary. The recess/resect procedure has the advantage that it
produces incomitance that allows fusion with a slight
Examination head turn. This more or less buys time while the
near overcorrection is resolving. The surgical treat-
Visual acuity with myopic correction showed
ment of convergence insufficiency should be under-
OD 20/20 and OS 20/20, near 20/20. Her glasses
taken only with the understanding that the postsurgi-
were OD -2.00 +0.50 X 180 degrees and OS -2.00
cal treatment course is complicated by varying peri-
+0.50 X 180 degrees. Prism and cover measurement
ods of diplopia. Before deciding on the type of sur-
at distance was 18 prism diopters of intermittent
gery, the surgeon must recognize that a bimedial rec-
exotropia and near 30 prism diopters of intermittent
tus resection produces a postoperative condition
exotropia. Near point of convergence was remote.
where, in the presence of an overcorrection, no head
The patient fused 8/9 stereo dots (50 seconds). There
posture can be assumed to avoid the diplopia.
was slight overaction of the inferior oblique muscles,
Diplopia after bimedial rectus resection must either
producing a small V pattern. The remainder of the
be treated early on with prism or by occluding one
eye examination was normal.
eye.
Diagnosis
Convergence insufficiency intermittent
exotropia.
Treatment
Near point of convergence push up exercises
can be helpful. Base-in prism is a possible short-term
treatment.
379
Chapter 16
Clinical picture
A B C
A, The eyes are aligned in the primary position; B, full abduction of the right eye; C, limited abduction of the left
eye
History Diagnosis
This 35-year-old woman had a resection of the Diplopia after recession-resection for intermit-
left medial rectus muscle and recession of the left lat- tent exotropia, presumably caused by a tight left
eral rectus muscle for what she described as an inter- medial rectus muscle.
mittent exotropia. Records from the previous surgery
were not available. She complains bitterly of diplop- Treatment/Surgery
ia that is worse when she looks to the left. It is very Recession of the left medial rectus muscle after
difficult for her to carry on her work as a bank teller forced duction testing to confirm restriction of abduc-
because of the diplopia. In order to maintain single tion.
binocular vision she must turn her head to the left and
maintain eyes to the right. Comment
Examination In a case of diplopia after surgery for intermit-
tent exotropia such as this where ductions are defi-
Visual acuity is 20/20 in each eye without cor- nitely limited either because of mechanical restriction
rection. The near point of accommodation is satis- or muscle weakness, prompt repeat surgery is indicat-
factory, and the patient could read 20/20 at near easi- ed. This is in sharp contrast to the overcorrected
ly. With 20 degrees of left face turn, 9/9 stereo acu- intermittent exotropia patient with perfectly free duc-
ity is recorded (40 seconds). Prism and cover testing tions who should be treated conservatively, often for
reveals 5 prism diopters of esotropia in the primary periods of months, with prism for the diplopia. The
position. The eyes are aligned in right gaze and 20 precise amount of surgery to be done in a case like
prism diopters of esotropia is found in left gaze. this cannot be determined arbitrarily on the basis of
Abduction of the left eye is moderately restricted. the deviation. In most cases a small recession of the
Saccadic velocity to the left is observed to be brisk previously resected muscle will suffice. This type of
and equal to saccadic velocity to the right. Passive case is ideally suited for an adjustable suture reces-
abduction of the left eye was noted to be moderately sion. Even after successful surgery such a patient
stiff. With a red lens over the right eye, the patient may be able to find diplopia by looking far to the
observed diplopia starting at 10 degrees of dextrover- left. In cases like this, I stress to the patient that the
sion and continuing through the primary position to surgery is a success and that they should remain sat-
all fields of left gaze. Single vision was present isfied as long as they must find the diplopia and the
beginning at 10 degrees of dextroversion and contin- diplopia does not find them!
uing to full dextroversion.
380
Strabismus case management
Clinical picture
A B
C D
Congenital Brown syndrome in left eye. A, The chin is up and pointing to the right;
B, downshoot of the left eye while attempting to look up and to the right; C, moder-
ate limitation of elevation left eye in upgaze; D, no limitation of elevation of the left
eye in abduction
History
Shortly after she started to walk, this 7-year- and is not limited in gaze up and to the left. The
old girl was noted by her parents to persistently keep remainder of the eye examination is normal. The
her chin up. The family also noticed that the left eye neck is supple with no apparent orthopedic cause for
looked different at times. This child has otherwise the head posture. Because of the young patients lack
been perfectly healthy with no ocular problems or of cooperation, it is impossible to do forced duction
systemic complaints. testing in the clinic. Finding a restriction on attempt-
ed passive elevation of the left eye in adduction
Examination would have confirmed the diagnosis of Brown syn-
During casual observation of this patient while drome and would have differentiated it from left
obtaining this history she assumes a head posture inferior oblique palsy. However, given the rarity of
with her chin pointing slightly up and to the right. inferior oblique palsy compared to Brown syndrome
Visual acuity is 20/20 in each eye. Stereo acuity is and the degree of limitation to elevation in straight
measured to 40 seconds (9/9 dots). During versions, up gaze, the presumptive diagnosis of Brown syn-
the left eye did not elevate in adduction. The left eye drome can be made with confidence. Of course, this
actually dips down below midline in dextroversion. must be confirmed by passive duction testing done in
Elevation is moderately limited in straight upgaze the operating room before surgery.
381
Chapter 16
Diagnosis
Congenital Brown syndrome. gested by Wright and is being done by many surgeons
with success.
Treatment/Surgery A major frustration in the surgical management
Exploration of the left superior oblique tendon, of Brown syndrome is that passive ductions may be
find and treat any restriction freed at the time of surgery only to become severely
restricted again in the postoperative period. Because
Comment of this, the eye may be sutured in the adducted ele-
Brown syndrome is best thought of as a vated position with a traction suture for several days
mechanical restriction to the full separation of the postoperatively. This apparently logical maneuver is
trochlea and the superior oblique insertion in eleva- not done often. In rare cases of Brown syndrome,
tion and adduction. It must be recognized that it can disinsertion of the posterior seven-eighths of the
have many causes and if it is to be cured, it will superior oblique tendon insertion has been effective.
require different treatments. Brown believed that an This only underscores the fact that this condition,
abnormally restrictive sheath around the superior which is really a physical sign rather than a disease
oblique tendon, the principal structure connecting the process, has multiple etiologies and should be expect-
trochlea and the insertion of the superior oblique ten- ed to have multiple remedies.
don into sclera was the cause of Brown syndrome. I now prefer to treat Brown syndrome after
Cutting the superior oblique tendon remains the most securing exposure of the entire tendon after a cuffed
effective way to relieve the restriction. In most cases superior limbal incision. This offers a good view of
this would solve the problem if the restriction is the anatomy and enables specific treatment of the
caused by a tendon or trochlear anomaly. The closer cause of the Brown (see chapter 6 ).
to the trochlea the superior oblique tendon is cut, the In young children with Brown syndrome, an
more likely the restriction is to be eliminated. indirect technique for performing the equivalent of
Postoperative superior oblique palsy occurs for the passive ductions is the differential intraocular pres-
same reason as the Brown is cured! About one-third sure test. A tonometer is used to record intraocular
of patients having superior oblique tenectomy for pressure in the primary position and also during
Brown syndrome have superior oblique underaction attempts at elevating the left eye in adduction. A
postoperatively and need inferior oblique weakening pressure rise of 5 to 10 mm Hg on attempted eleva-
as a second procedure. For this reason, I do not rec- tion indicates that the inferior oblique is contracting
ommend inferior oblique weakening at the same pro- in the face of a nonyielding antagonist, implying a
cedure as superior oblique tenectomy in treating mechanical restriction to elevation. If no pressure
Brown syndrome. Use of a silicone expander to rise occurs, a paresis of the inferior oblique is
lengthen the superior oblique tendon has been sug- inferred (see p.103).
382
Strabismus case management
Clinical picture
A B C
D E
Acquired Brown syndrome of the right eye with limitation of elevation in adduction.
A primary position, eyes are aligned D normal upgaze
B elevation of the right eye limited in gaze up to the left E normal downgaze
C normal motility up and to the right
History
This 36-year-old woman noted the sudden right eye suddenly became stuck and would not ele-
onset of diplopia only when looking up 4 months vate in adduction. At this time a soft nontender mass
ago. The diplopia typically lasted for a few hours could be felt just below the trochlear area at the inner
and then went away. It recurred on a daily basis and aspect of the junction of the medial and superior
now happens several times a day. The double vision orbital rim.
is associated with a full feeling in the corner of the
right eye, and she has heard a click and felt a rub Diagnosis
in the corner of the right eye during these episodes of Acquired Brown syndrome, probably cyst of
double vision. No other health problems are evident. superior oblique tendon.
Examination Treatment/Surgery
The initial eye examination was normal: 20/20 Exploration of the superior oblique tendon
vision in each eye, stereoacuity at 9/9 dots (40 sec- after a cuffed limbal incision with excision of the
onds), and orthophoria is seen in the primary posi- cyst.
tion. After several minutes of testing versions, the
383
Chapter 16
Comment
This case describes just one of many causes of palsy are so disappointing, it is better to leave these
acquired Brown syndrome (see chapter 9) Other patients untreated, provided the symptoms are not too
somewhat similar cases that I have seen have been great. The Brown syndrome caused by trauma to the
associated with a painful or at least tender spot in the trochlear area tends to recur after surgery, producing
area of the trochlea with a constant or intermittent poor surgical results. Iatrogenic Brown syndrome
limitation of elevation. Tenderness associated with a also occurs after too large a tuck or resection of the
feeling of induration on palpation suggests inflamma- superior oblique tendon. At reoperation to take down
tion. I have treated several such patients with injec- a tuck or recess a previously resected tendon, the
tion of soluble steroid in the area of the trochlear cuff amount of adhesions and therefore the difficulty of
but not into the trochlea itself. Several patients have exposure will depend largely on the care and preci-
required repeated injections, but all cases have sion of the original surgery. If the surgery had been
resolved. done primarily on the temporal side, near the superi-
Acquired Brown syndrome from trauma to the or oblique insertion, the reoperation is much easier.
area of the trochlea and the superior oblique tendon However, when significant scar and reaction is found
presents a formidable therapeutic challenge. Because in the area of the tuck, a tenectomy can be done with
results of treatment of this and what has been called good results. It is best to avoid tucking the superior
canine tooth syndrome or Class VII superior oblique oblique tendon medial to the superior rectus.
Clinical picture
A B C
A 55-year-old woman with iatrogenic Brown syndrome of the right eye after superior oblique resection for right
superior oblique palsy. A, Eyes are aligned in primary position; B, right eye shows limited elevation in adduc-
tion; C, normal motility up and to the right.
Comment
This 55-year-old woman had right superior able to carry out her work as a cashier in a school
oblique resection and right superior rectus recession cafeteria symptom free. She is able to notice diplop-
for class IV acquired superior oblique palsy (see p. ia looking up and to the left, but she has no difficulty
155). In spite of a small (6.0 mm) superior oblique avoiding this field. Other patients with less Brown
tuck, iatrogenic Brown syndrome of the right eye is syndrome after superior oblique shortening have
evident. Before the superior oblique resection, this complained bitterly to the point of being incon-
patient had 30 prism diopters of right hypertropia in solable. This points out the fact that tolerance of
left gaze and slightly less in down right gaze. After patients to minor inconveniences in the postoperative
surgery the eyes were aligned in the primary position period varies greatly. For this reason, all of these
and less than 5 prism diopters of intermittent right patients should be counselled thoroughly before sur-
hypertropia was measured in downgaze. The patient gery. Iatrogenic Brown syndrome like this also tends
is extremely pleased with the results of surgery and is to lessen with time (see p. 395).
384
Strabismus case management
Clinical picture
A B C
A, Minimal narrowing of left palpebral fissure on right gaze; B, slight left esotropia in primary posi-
tion; C, limited abduction of the left eye; D, left face turn and right gaze to achieve comfortable sin-
gle binocular vision.
History Treatment/Surgery
This 7-year-old boy was presented for exami- Recession of the left medial rectus.
nation by his parents because his first grade teacher
said that the boy turned his head constantly. In retro- Comment
spect, the parents realized that they had also noted These patients can do well with surgery.
this behavior but had not thought it to be significant. However, it is necessary to tell the patient and/or the
family that the main reason for the surgery is to
Examination improve head posture and allow the patient to have
The patients slight left face turn presents a typ- straight eyes in the primary position with a straight
ical picture that can be diagnosed at once by the expe- head. Abduction will not be improved. For this rea-
rienced observer. With the head straightened, the left son, some surgeons suggest placing a posterior fixa-
eye becomes slightly esotropic. The left palpebral fis- tion suture on the contralateral (right in this case)
sure narrows somewhat on dextroversion and the left medial rectus. In mild esotropic Duane syndrome
eye fails to abduct fully on levoversion. The remain- (class I), some surgeons have recommended transfer-
der of the eye examination is normal. Visual acuity is ring the superior an inferior recti to the lateral rectus.
20/20 in each eye, retinoscopy after 1% Cyclogyl is I have not done this and do not recommend it because
OD +1.00, OS +1.00, and Stereo acuity, with left face of the risk of producing and exotropic Duane (class
turn and eyes right, is at 9/9 dots (40 seconds). II) with severe enophtahlmos on attempted adduction
postoperatively and also the possibility of creating a
Diagnosis vertical deviation in the primary position. Class I
Duane syndrome with esotropia (class I). Duane syndrome and any of the types discussed sub-
sequently can be bilateral. In the case of bilateral
involvement, appropriate surgery is done on both
eyes.
385
Chapter 16
Clinical picture
B C
A, Primary position eyes are slightly exotropic; B, slightly limited abduction; C, limited
adduction with narrowing of th left palpebral fissure
History Diagnosis
This 10-year-old girl had no trouble with her Duane syndrome with exotropia and limited
eyes, but her parents noted that at times her eyes did adduction left eye (class II).
not seem to work together and that she turned her face
to the right when reading. The left eye is also seen to Treatment/Surgery
shoot up. This patient may benefit from a right lateral
rectus recession alone. If the upshoot is a problem,
Examination both the medial and lateral recti may be recessed. If
Observing this girl it is evident that she has a it is believed that this will produce too much exode-
10-degree right face turn. Testing of versions con- viation, the lateral rectus of the sound eye may be
firms reduced adduction of the left eye with narrow- recessed.
ing of the palpebral fissure and enophthalmos.
Upshoot of the left eye on adduction was observed. Comment
Visual acuity is 20/20 in each eye. Retinoscopy after The principal aim of this surgery is to achieve
1% Cyclogyl is OD +1.50 and OS +1.00 +.50 X 80 normal head posture, maintain aligned eyes in the pri-
degrees. The remainder of the eye examination was mary position, and reduce upshoots and downshoots
unremarkable. The stereo fly test indicated gross without disrupting fusion. Success can be measured
stereopsis (3000 sec). as a factor of how well these goals are met.
386
Strabismus case management
B C
D E
A, In primary position, the eyes are aligned; B, left eye adduction is slightly limited with mild fissure
narrowing on the left; C, left eye abduction is moderately limited; D, elevation is intact; E, depres-
sion is intact.
History Diagnosis
This 15-year old boys left eye has always Duane syndrome with straight eyes and limited
looked funny, according to the family. He reports abduction and adduction (class III).
frequent double vision. At times the left eye seems to
go out of sight. Treatment/Surgery
Examination Recession of the left medial and left lateral rec-
tus muscles, or no surgery.
Visual acuity is 20/20 in each eye and
stereoacuity is 6/9 dots (80 seconds). Both abduction
and adduction are moderately limited. On extreme
attempts to adduct the left eye, it shoots up under the
upper lid and the pupil cannot be seen. If the eye
attempts to adduct while looking slightly below mid-
line, the eye shoots down but the cornea is less
obscured by the lower lid.* The eyes are aligned in
the primary position as the patient assumes a slight
right face turn.
*The up and down shoot are not shown in the clinical photographs. Actually this never occurs unless the patient makes it happen. The
patients main complaint is a feeling of tightness of the left eye.
387
Chapter 16
Comment
The principal reason for doing this surgery is to is also recessed. The three recession procedure is not
reduce the enophthalmos and the upshoot (and down- commonly done but with this rare indication it is
shoot). Weakening the two opposing rectus muscles effective.
of the involved eye is designed to accomplish this To lessen the up- and downshoot associated
without adversely affecting the primary position with this type of Duane syndrome,Y splitting of the
alignment. This type of Duane syndrome with severe insertion of the lateral rectus muscle of the involved
enophthalmos and up- and downshoots can also have eye may be done, with or without recession. Another
an esotropia in the primary position. If this is the surgical treatment is to do a posterior fixation suture
case, recessing the opposing horizontal recti will on the lateral rectus of the involved eye. Both this
mean that the esodeviation in the primary position and the Y split are intended to keep the lateral rec-
persists. When this type of patient is encountered, in tus from slipping above or below the horizontal plane
addition to recessing both horizontal recti in the of the lateral rectus where it becomes either an ele-
involved eye, the medial rectus of the uninvolved eye vator or depressor.
B C
This 6-year-old girl has a more severe manifestation of straight-eyed Duane syndrome or
class III according to the Huber classification. She demonstrates the disappearing eye or
the pumpkin seed sign where the eye disappears usually beneath the upper lid on adduc-
tion. A, The eyes are aligned in the primary position; B, abduction in the left eye is limited;
C, during adduction the palpebral fissure narrows markedly and the left eye shoots up
under the lid because of the knife edge created by the nonyielding left lateral rectus in
response to the vigorous contraction of the left medial rectus.
388
Strabismus case management
Clinical picture
A B
History Comment
This 18-month-old boy was noted by his par- This type of patient is very unusual; I have seen
ents to have widely divergent eyes that never worked only a handful in the past 40 years. The reason for the
together from birth. The child was also diagnosed as simultaneous abduction seems to be misdirection of
having arthrogryposis multiplex congenita at age 1 the third nerve fibers from the medial rectus, which
year. innervate the lateral rectus, as is known to occur in
other Duane syndrome patients. Added to this condi-
Examination tion is the fact that the involved eye is so far in abduc-
This child had stiff joints, especially in the tion that when the co-contraction is initiated, the bal-
hands. Vision appeared to be normal in each eye. An ance of forces is tipped over toward the lateral rectus,
exotropia of 50 prism diopters was present in the pri- which overcomes the opposing effort of the medial
mary position with left eye fixation. On gaze to the rectus contraction. Repositioning the muscles at sur-
left the exotropia reduced to approximately 40 prism gery has no effect on the innervation pattern, meaning
diopters. On right gaze the exotropia increased to that after alignment is obtained, if indeed this is even
more than 100 prism diopters because the left eye possible, the problems of the co-contraction will
abducted at the same time the right eye abducted in remain. However, if the exotropia is reduced sub-
the way it was expected to while looking to the right. stantially, simultaneous abduction should not occur
simply because of alteration of the mechanical lever
Diagnosis arm that is taken away in this case from the left later-
Duane syndrome with simultaneous abduction al rectus. This type of Duane syndrome with simul-
(perversion of the extraocular muscles) (class IV). taneous abduction could be confused with congenital
third nerve palsy on the basis of the large exotropia.
Treatment/Surgery Several points of difference include absence of ptosis
and normal pupil response. In addition, simultaneous
Recession of both lateral recti greater on the left abduction is not a part of third nerve palsy, even with
with resection of the left medial rectus. misdirected regeneration of the third nerve fibers.
389
Chapter 16
Clinical picture
B C
A, The head is tilted left with slight chin depression. B, On upgaze to the left, moderate over-
action of the right inferior oblique can be seen. C, Motility is normal on gaze up and to the
right. Moderate facial asymmetry is characterized by a fuller face on the right.
History Examination
This 34-year-old woman complained of vertical Visual acuity is right eye 20/30-2 and left eye
diplopia that was better if she tilted her head to the 20/40 corrected to 20/20 in each eye with -0.75
left and tipped her chin down. She is now concerned sphere. Versions show 2+ overaction of the right
because it is becoming more difficult for her to work inferior oblique with normal action of all other mus-
for long periods at a computer, a task that her job cles. Stereo acuity was 9/9 dots (40 seconds). Prism
requires. and cover testing shows 10 prism diopters of right
hypertropia in the primary position, increasing to 14
prism diopters of right hypertropia in left gaze. A
small right hypertropia is measured in right and
downgaze, while 4 prism diopters of right hypertropia
is noted in upgaze. With the Bielschowsky head tilt,
the right hypertropia increases to 16 prism diopters on
right head tilt and decreases to 4 prism diopters right
hypertropia on head tilt to the left. No cyclotropia is
noted with double Maddox rod test. The remainder of
the eye examination is normal.
390
Strabismus case management
Diagnosis
Class I right superior oblique palsy. oblique myectomy. Although it is likely that this is a
congenital superior oblique palsy because of the lax
Treatment/Surgery superior oblique traction test and absence of history
Right inferior oblique myectomy. for trauma, this type of picture, but with a normal
superior oblique tendon on traction testing, is seen
Comment very commonly in acquired superior oblique palsy. It
should be strongly emphasized here that the safest
This woman demonstrates a class I superior
surgical procedure for patients with superior oblique
oblique palsy producing a moderate-sized, incomitant
palsy is a weakening procedure of the antagonist infe-
right hypertropia manifesting principally as overac-
rior oblique. It is difficult to go wrong when this pro-
tion of the right inferior oblique. Traction testing of
cedure is done properly. Treatment other than an
the superior oblique at surgery demonstrated a lax
inferior oblique weakening in superior oblique palsy
superior oblique tendon on the right and a normal ten-
should be undertaken only after carefully analysis of
don on the left. Because of the small angle and min-
all measurements. These include especially the diag-
imal superior oblique findings as well as the fact that
nostic position prism and cover measurements and
the superior oblique had normal function and the infe-
the intraoperative superior oblique traction test find-
rior oblique overaction was the main motility prob-
ings.
lem, this patient was best treated with a right inferior
391
Chapter 16
Clinical picture
History Diagnosis
This 87-year-old man began to note double Class II acquired right superior oblique palsy.
vision 6 weeks earlier. Images were separated verti-
cally, causing him difficulty in reading the newspaper Treatment
and in watching television. He also stopped driving Fresnel prism, 6 diopters base-down, right eye.
his automobile, which he used only for short daytime
trips in a familiar area. He states that he takes some Surgery
blood pressure medication prescribed by his internist
None.
and that his blood pressure is satisfactorily con-
trolled. When the double vision started, this man was Comment
referred to a neurologist by his internist. A CT scan
and MRI of the head were said to be normal. Lumbar This is a typical pattern for acquired superior
puncture findings, blood sugar testing, and EEG were oblique palsy resulting from a presumed microvascu-
also within normal limits for a man of his age. When lar accident. These patients usually do very well
all of these tests were found to be in the range of nor- with temporary prism. The deviation often resolves
mal, the man was referred to a general ophthalmolo- completely and prism therapy can be discontinued.
gist, who then referred the patient to me. If this does not happen, permanent prism can be
given. The extensive work-up in this case was not
Examination necessary. Such an exercise should be avoided on
the basis of the unnecessary expense and the incon-
Visual acuity with correction is OD 20/40 and
venience to the patient. A curious thing happened
OS 20/30. Slight lens opacities were thought to
when the patients son was told that his father had
account for the decrease in vision. Prism and cover
superior oblique palsy. Is that like a trochlear
measurements were 6 prism diopters right hyper-
palsy? the son asked. This mans son had looked up
tropia in the primary position, 4 prism diopters in up
the symptoms his father complained of in the Merck
left gaze, and 10 prism diopters hypertropia in down
Manual* and had arrived at the proper diagnosis. A
left gaze. Slight underaction of the right superior
similar level of clinical acumen on the part of the
oblique is noted on testing of versions. The right
patients internist and neurologist would have spared
inferior oblique did not overact. Five degrees of
the patient a great deal of expense and inconven-
excyclotropia of the right eye is seen on testing with
ience.
the double Maddox rod. Blood pressure in the left
arm was 134/88.
*Berkow R, editor: The Merek Manual of Diagnosis and Therapy, ed 15, Rathway, NJ, 1987, Merck Sharp & Dohme. Today, the son
would have obtained this kind of information on the Internet.
392
Strabismus case management
Clinical picture
A, Abnormal head posture with the head tilted to the left and the chin depressed. The face
is fuller on the right. B, The Bielschowsky head tilt test is positive for a right superior
oblique palsy, as indicated by increased right hypertropia on right head tilt when compared
to left head tilt. C, There is moderate underaction of the right superior oblique compared to
the left superior oblique which appears to overact with slight underaction of the right inferior
rectus.
393
Chapter 16
History
This 36-year-old woman has been bothered by
a deviating right eye since early childhood. She has
had diplopia for as long as she can remember but was
able to tilt her head to relieve it. She has worn prism
glasses for many years. Her friends and associates at
work comment on the fact that she tilts her head con-
stantly.
Examination
Visual acuity with correction is OD 20/30-2 and OS
20/20. Glasses are OD -1.00 +0.50 X 60 degrees and
OS -1.00 X 100 degrees with 3 diopters of base-
down prism in the right lens. Prism and cover meas-
urements are 20 prism diopters of right hypertropia in
the primary position, increasing to a maximum of 35
prism diopters in up left gaze and 38 prism diopters
right hypertropia in down left gaze. Five degrees of long redundant right superior oblique tendon was
excyclotropia is measured with the double Maddox found. This was associated with a -4 (loose) superi-
rod. With the Bielschowsky head tilt test, there is or oblique traction test on the right compared with a
right hypertropia of 40 on right tilt and 15 on left normal superior oblique traction test on the left. This
head tilt. There is 2+ overaction of the right inferior type of patient does well with a tuck or resection of
oblique and 2+ underaction of the right superior the superior oblique tendon. This is in contrast to the
oblique. Stereoacuity is nil. The remainder of the usual acquired superior oblique palsy patient who,
examination is normal. because of an anatomically normal superior oblique
tendon is much more likely to have a Brown syn-
drome after tuck or resection of the superior oblique
Diagnosis tendon. I avoid tuck or resection of the superior
oblique tendon in an acquired superior oblique palsy.
Superior oblique palsy, class III, probably con- This patient postoperatively had a moderate limita-
genital. tion of elevation in adduction in the right eye (Brown
Treatment/Surgery syndrome), but it did not bother her except when
looking up and to the left, a field of gaze that she can
Right superior oblique resection, right inferior easily avoid. Her stereoacuity was nil preoperative-
oblique myectomy. ly. It improved to fusion of the stereo fly (3000 sec-
onds) after surgery. The patient had a small residual
Comment intermittent right hypertropia measuring 6 to 8 prism
This patient has a classic congenital superior diopters in the primary position, but she controlled
oblique palsy persisting into adulthood. At surgery, a this easily and without symptoms.
A B
A, Viewed from below, the superior oblique traction test of the right eye is -4; that is, the globe is pushed back in the
orbit with only a faint tactile evidence of the band formed by the superior oblique tendon between the trochlea and the
insertion.. B, In contrast, the left superior oblique traction test is normal. A band of superior oblique tendon could be
felt. Note that the cornea is still visible on the normal left side (see p. 97).
394
Strabismus case management
Clinical picture
A B
History
This 52-year-old woman sustained closed head
trauma 24 months earlier in a motor vehicle accident.
Since that time, she has been bothered by constant
vertical diplopia. She is wearing heavy prism in her
glasses. She wants to be rid of the prism and seeks
treatment for her vertical diplopia.
Examination
Visual acuity with correction is right eye 20/20
and left eye 20/20 while wearing OD +2.00+1.50 X
22 degrees and OS +1.00+2.00 X 165 degrees. There
is 9 diopters of base down prism in the right lens and
8 diopters of base-up prism in the left lens. The glass-
es are physically very heavy. Prism and cover testing
shows 25 prism diopters of right hypertropia in the
primary position increasing to 30 in left gaze and
decreasing to 18 in right gaze. The right hypertropia Diagnosis
is 24 in down left gaze and 22 in down right gaze.
Eight degrees of right excyclotropia is measured. The Class IV acquired right superior oblique palsy.
right hypertropia increases with right head tilt. Two
plus overaction of the right inferior oblique and 1+ Treatment/Surgery
underaction of the right superior oblique are noted. Right superior oblique resection, right superior
The patient fuses 6/9 stereo dots (80 seconds) with rectus recession.
her prism glasses.
395
Chapter 16
Comment
This patient had a spread of comitance because before I recognized the value of the preoperative
she had contracture of the right superior rectus mus- superior oblique traction test. Now I would avoid
cle. This caused the right eye to hang up in down even a small superior oblique resection with a normal
right gaze. The surgery done on this patient was tendon weakening the ipsilteral inferior oblique
aimed at decreasing the hypertropia in left gaze by instead. Another significant postoperative event
resecting the right superior oblique tendon and occurred with this patient. Two days after surgery the
decreasing the hypertropia in right gaze by recessing patient called the office distraught, saying that her
the presumably tight right superior rectus. A small, eyes were worse than before surgery and that she was
6.0 mm resection of the right superior oblique tendon sorry she had the operation. The patient was, of
was done. This muscle was chosen for resection course, seen immediately. We were reminded to our
instead of weakening the right inferior oblique chagrin of the 17 diopters of prism (to correct the
because strengthening the superior oblique would be right hypertropia) in her glasses, which she had to
more likely to eliminate the 8 degrees of excyclotor- wear in order to see! Since the surgery had reduced
sion. However, weakening the antagonist inferior the vertical deviation to a small intermittent right
oblique would be a reasonable choice. The incyclo- hypertropia, the preoperative prisms were creating
torsion effect of strengthening the right superior diplopia! Our policy now is to send such patients
oblique is greater than the excyclotorsion that would home from surgery with Fresnel prism to temporarily
be produced by recessing the superior rectus. This offset permanent prism in the glasses that were appro-
case stresses the importance of carefully assessing the priate preoperatively. In this patient, we were guilty
diagnostic position measurements in a superior of an oversight. The surgeon and staff should be sure
oblique palsy in order to design a surgical procedure to make provisions to nullify the unneeded prism by
to create postoperative comitance. Postoperatively adding to the patients present glasses offsetting
this patient had a mild iatrogenic Brown syndrome Fresnel prism for wear in the immediate postopera-
but she was not bothered by it. This case was done tive period (see p. 384).
396
Strabismus case management
Clinical picture
A B
C D
E F
With her chin down and looking up, this patient is able to nearly fuse. A slight horizontal
diplopia persists. A definite V pattern is present. Both superior obliques underact but
more so on the left. A, Chin down in primary position; B, exotropia in upgaze; C, normal
up-right gaze; D, normal up left gaze; E, esotropia in downgaze (V pattern); F, underac-
tion of the left superior oblique.
History
This 28-year-old woman was involved in a right head tilt 16 prism diopters of esotropia and 2
motor vehicle accident 3 1/2 years before being seen prism diopters of right hypertropia is noted. On left
by us. She had been comatose for 3 days after the head tilt, 16 prism diopters of esotropia and 4 prism
accident. Since the time she regained consciousness, diopters of left hypertropia is noted. With a double
she has had diplopia. The only relief for the diplopia Maddox rod, 17 degrees of excyclotropia is measured
is to patch one eye. The images are closer together in the primary position. Versions show moderate
when she puts her chin down and looks upward, but underaction of the right superior oblique and marked
even then some horizontal diplopia persists. At times, underaction of the left superior oblique with no over-
images appear tilted. action of the inferior obliques. The remainder of the
eye examination is unremarkable.
Examination Prism and cover testing in the nine diagnostic
Visual acuity without correction is right eye positions is very difficult in bilateral superior oblique
20/25-2 and left eye 20/20. Prism and cover test in palsy and is actually unnecessary either for diagnosis
the primary position shows 10 prism diopters of or for planning treatment. The measurements shown
esotropia in the distance and 12 prism diopters of above are sufficient.
esotropia at near. In right gaze the esotropia is 10
prism diopters and in left gaze it is 16 prism diopters
with 10 prism diopters of right hypertropia. With
397
Chapter 16
Diagnosis
Bilateral superior oblique palsy. therapeutic challenge since these conditions are often
associated with severe head trauma, which in turn can
Treatment/Surgery lead to central disruption of fusion and produce a sit-
Bilateral inferior rectus recession 5.0 to 6.0 mm uation where the patient may have a secondary devi-
combined with anterior and temporal transposition of ation in all fields of gaze.
the anterior half of the superior oblique insertion is a Strengthening of the superior oblique has its
first choice for treatment. Do not tuck the superior own unique problems related to the special anatomy
oblique tendons; this will cause a bilateral Brown. of the superior oblique tendon. I recently did a bilat-
Some would weaken both inferior obliques. The eral superior oblique tuck in a 4-year-old girl with a
medial recti could be shifted down without recession. V pattern, a chin-down posture, and 4+bilateral
inferior oblique overaction. The usual diagnosis in
Comment this type of case is primary overaction of the inferi-
This patient demonstrates the typical clinical or obliques. At surgery, this child had bilateral -4
findings of bilateral superior oblique palsy. These (very loose) superior oblique traction tests.
include a history of closed head trauma, spontaneous Postoperatively she continued to manifest a fairly
torsional diplopia, V pattern, and reversing large V pattern and bilateral inferior oblique weak-
Bielschowsky test. Bilateral superior oblique palsy ening was required. As a contrast in other cases of
represents a serious therapeutic challenge. The safest bilateral superior oblique palsy after trauma, small
initial procedure is to do a bilateral inferior rectus bilateral superior oblique tucks have caused trouble-
recession. This will help to open up the V and some Brown syndrome and have resulted in only a
should have some beneficial effect on the cyclotropia. small window of single binocular vision.
However, in this patient, with no overaction of the In some patients with bilateral superior oblique
inferior obliques, the result of inferior oblique weak- palsy one side can be masked by a greater deviation
ening might be disappointing. Tucking of a superior on the other side. Patients who have atypical unilat-
oblique in an acquired case presents the risk of pro- eral superior oblique palsy findings, including a head
ducing a Brown syndrome. A bilateral anterior and tilt test that shows a moderate hyperdeviation on tilt
temporal shift of the anterior fibers of the superior toward the involved side and no or almost no devia-
oblique may be the best treatment in that it gives the tion on tilt toward the other side, with a history of
patient the opportunity to be free of cyclodeviation head trauma and cyclodiplopia should be suspect for
with only minimal risk of a postoperative Brown. In masked bilateral superior oblique palsy. If bilateral
this patients case, since there is esotropia even in masked superior oblique palsy is suspected, surgery
upgaze, she has no place to fuse! Some cases of bilat- may be done as for bilateral superior oblique palsy,
eral superior oblique palsy are able to fuse in far with the provision that more surgery per muscle or
upgaze, which tends to give a better starting point for more muscles be treated on the more affected side.
obtaining a bifoveal fusion result in a useful field. As an alternative, the more involved side can be treat-
Bilateral superior oblique palsy, like bilateral ed at the initial procedure and the less involved side
sixth nerve and third nerve palsy, presents a special treated appropriately at a second procedure.
398
Strabismus case management
Clinical picture
A B
This patient has canine tooth syndrome A, the eyes are aligned in the primary position, note the diagonal
scar across the forehead ending at the trochlea. B, limited elevation of the left eye in adduction. C, normal
elevation of the left eye in abduction
History Examination
This 11-year-old boy was attacked by a large Visual acuity is 20/20 in each eye. Cycloplegic
German Shepherd dog. He was bitten around the left retinoscopy is plano. With slight chin depression
eye. Repair of the skin was carried out immediately stereo acuity is 9/9 (40 seconds). On testing of ver-
after the injury. When the swelling around the left sions, movement of the left eye is limited in both ele-
eye subsided, the boy noted that things looked double vation and depression in adduction. In addition, the
when he looked up or down. intraocular pressure in the left eye is more than 10
mm Hg elevated in the left eye compared to the right
eye on attempts to elevate and depress the eye, imply-
ing restriction of eye rotation in the face of normal
generated force. The remainder of the eye examina-
tion is normal.
399
Chapter 16
Diagnosis Comment
Canine tooth syndrome: class VII superior This condition, first described by Philip Knapp,
oblique palsy. can occur after trauma in the area of the superior
oblique tendon and trochlea and affect eye movement
Treatment/Surgery on a strictly mechanical basis. In other cases it can be
Surgery could be done depending on the associated with superior oblique palsy, plus mechani-
patients symptoms. If surgery is elected, an attempt cal limitation of elevation from iatrogenic causes or
could be made to free the adhesions around the supe- from the trauma that directly or indirectly caused the
rior oblique tendon. A tenectomy of the superior superior oblique palsy. The mechanical limitation to
oblique near the trochlea (producing superior oblique elevation in adduction (Brown syndrome) is easiest to
palsy) and a weakening of the antagonist inferior eliminate when it has occurred after a tuck or resec-
oblique (treating superior oblique palsy) is a treat- tion of the superior oblique (iatrogenic Brown syn-
ment option. If this does not treat the palsy com- drome). In the case described here, the patient
pletely, a recession of the yoke inferior rectus could retained excellent fusion in the primary position and
be added. If the patient does well in the primary posi- did not want surgery. Attempts at improving motility
tion, no treatment may be indicated. by means of surgery could make such a patient worse
and should therefore be avoided unless a very trou-
blesome head posture is adopted or if diplopia is trou-
blesome.
400
Strabismus case management
Clinical picture
A B
A, Pronounced underaction of the left superior oblique on testing versions in a patient who at surgery was found to
have no left superior oblique tendon. B, A patient with Crouzon anomaly demonstrating marked overaction of the left
inferior oblique had bilateral absence of the superior oblique tendons confirmed at surgery.
History Treatment/Surgery
Both of the patients shown were known to have When no superior oblique tendon is found at
extreme vertical deviations which were variable. surgery, it is necessary to weaken and strengthen
They were brought for examination by their parents available vertically acting muscles to produce the
because of this. most favorable alignment. The following sequence
for extraocular muscle surgery is logical: (1) Weaken
Examination the antagonist inferior oblique, (2) weaken the yoke
When a patient with presumed congenital supe- of the absent superior oblique (contralateral inferior
rior oblique palsy is seen with pronounced underac- rectus), (3) weaken the ipsilateral superior rectus, and
tion of the superior oblique, this should raise suspi- (4) strengthen the contralateral superior rectus. To
cion that the superior oblique tendon could be absent. this scheme could be added horizontal rectus surgery
Two signs that point to the possibility of this occur- of sufficient amount to treat any horizontal strabis-
ring in a patient with congenital superior oblique mus. It is usually sufficient to operate on only two
palsy are: horizontal strabismus, and amblyopia. vertical muscles at the first procedure.
Facial asymmetry is a nearly constant finding in
patients with congenital superior oblique palsy with
Comment
an anomalous tendon. Patients with severe craniofa- Patients with absence of one or both superior
cial anomalies such as patients with Crouzon anom- oblique tendons usually do not have normal bifoveal
aly shown here, are more likely to have absence of the fusion even with head tilt. This is in contrast to most
superior oblique tendon if they manifest superior acquired and those less severely affected congenital
oblique palsy signs. As is so often the case, absence superior oblique palsy patients, who usually have
of the superior oblique tendon was encountered ini- bifoveal fusion when they assume appropriate head
tially in this type of worst case scenario. Later, posture.
absence of the superior oblique tendon was noted in
less severely affected patients. In addition to being
more likely to have a superior oblique tendon anom-
aly, patients with congenital superior oblique palsy
frequently have facial asymmetry. The fuller face is
on the involved side. This occurrence is not fully
explained, but it seems the facial asymmetry is caused
by postural factors; i.e., the chronic head tilt.
401
Chapter 16
Clinical picture
B C
Left hypotropia greater in upgaze also with retraction of the left upper lid, Graefes sign.
A, Left hypotropia fixing with the right eye. B, The left eye depresses more than the right
eye on downgaze. C, Elevation of the left eye is limited.
History Diagnosis
This 36-year-old airplane mechanic began Euthyroid Graves (thyroid) ophthalmopathy
noticing vertical diplopia 6 months ago. At the begin- involving the left inferior rectus.
ning, he was able to see one object by raising his chin
to look downward. This strategy is no longer effec- Treatment/Surgery
tive and his double vision is constant. He is otherwise Recession of the left inferior rectus muscle with
in good health except for exogenous obesity (67 inch- (or without) an adjustable suture with smaller reces-
es tall, 280 pounds). He has no signs of hyperthy- sion of the right inferior rectus if any restriction is felt
roidism, and none were found earlier during routine at the time of surgery.
laboratory tests obtained by his internist.
Comment
Examination
In a patient like this, who has Graves (thyroid)
Visual acuity is 20/20 in each eye without cor- ophthalmopathy with the fellow eye apparently unin-
rection. Vertical diplopia is experienced in all fields. volved, recession of a single inferior rectus muscle
The left eye is hypodeviated approximately 20 prism can be very effective. On the other hand, if the fellow
diopters in the primary position. This appears to be a inferior rectus is restricted, even minimally, and only
larger deviation because of contraction of the left the more involved inferior rectus is recessed, the
upper lid. When attempting to look up, the left eye operated eye may become hypertropic postoperative-
lags behind the right eye. The left hypotropia persists ly with weakness of depression. This occurs because
in downgaze but is of lesser magnitude. Ductions are the eye with the recessed inferior rectus is subjected
normal in the right eye. to the efforts of a secondary deviation when the
After the conjunctiva of the left eye was anes- patient fixes with the unoperated but mildly restricted
thetized with proparacaine hydrochloride, passive fellow eye. When thyroid ophthalmopathy is bilater-
ductions were done and found to be severely restrict- al, even though the involvement is minimal in the less
ed to elevation in the left eye. Intraocular pressure involved eye, recession should be done in both eyes
was 17 mm Hg in both eyes in downgaze. The pres- to avoid progressive overcorrection after inferior rec-
sure in the left eye rose to 40 mm Hg on attempted tus recession.
upgaze, while the pressure in the right eye rose only
slightly (to 20 mm Hg in upgaze.)
402
Strabismus case management
A B
C D
Postoperative slippage of the left inferior rectus. A, Ten diopters left hypertropia in
primary position with ptosis of the left lower lid; B, limited depression of the left
eye. C, Reading is difficult because of increasing vertical diplopia from left hyper-
tropia in downgaze. D, Single binocular vision is possible in upgaze.
A B
C D
Schematic representation of the mechanism of progressive overcorrection after inferior rectus recession in a patient with
unequal bilateral inferior rectus thyroid ophthalmopathy. The + and - signs represent innervation (+) and relaxation (-).
A, Unilateral inferior rectus restriction (left eye) preoperatively. B, Corrected with recession of the restricted inferior rectus.
C, Bilateral unequal inferior rectus restriction preoperatively.. D, Postoperative slippage of the recessed left inferior rectus
caused in part by excess innervation to its antagonist, the left superior rectus by Herings law.
403
Chapter 16
History
When first seen, this 72-year-old woman had ophthalmopathy this is not always the case. Several
swelling around the eyes, redness, slight prominence obstacles to the ideal treatment occur. First and most
of her eyes, and diplopia that had been gradually important is the fact that the muscle operated on is not
increasing for the past year. When she watched tele- the only muscle involved. It is merely the most
vision or rode in a car, images were separated verti- involved muscle. This autoimmune disease undoubt-
cally. She closed one eye to read or she placed an edly involves all of the extraocular muscles but to a
occluder over the lens of her glasses. She stopped different degree. A CT scan or an MRI of the orbit
driving because of the diplopia. She had been treated routinely shows thickening of all of the muscles, not
with ablation of the thyroid by her internist, who just one or two. This means that if one muscle is
states that her condition is now stable, requiring only weakened, it may be transformed from being the
maintenance thyroid supplement. Visual acuity with stiffest muscle to the most lax, with another taking its
pseudophakic correction is 20/30 in each eye. Prism place as the stiffest muscle. As in the case described,
and cover testing in the primary position revealed 16 it is often the other inferior rectus that becomes the
prism diopters of left hypotropia (right hypertropia). stiffest muscle. If that eye then takes up fixation after
This decreased slightly on downgaze and increased the other inferior rectus has been recessed, the basis
slightly on upgaze. Intraocular pressure in the left for a secondary deviation may be present when the
eye was 16 mm Hg in the primary position and superior rectus, the antagonist of the surgically weak-
increased to 30 mm Hg on attempted upgaze. In the ened left inferior rectus, receives extra innervation. It
right eye only a 4 mm Hg pressure rise was recorded is being stimulated equally with the superior rectus of
in upgaze. With a 10-diopter prism held base up in the fellow eye, which now has as its antagonist a stiff
front of the left eye, the patient could see 4/9 stereo inferior rectus muscle. This in turn destabilizes the
dots on the Titmus test (140 seconds). Observed sac- newly reattached inferior rectus. This sequence of
cadic velocity during elevation of the left eye was events leads to an overcorrection (hypertropia), espe-
equal to that of the right. She was diagnosed as hav- cially if an adjustable suture has been used. For this
ing stable thyroid ophthalmopathy, and an adjustable reason it is important to balance forces when weak-
left inferior rectus recession was done. The eyes were ening a muscle, especially the inferior rectus, in treat-
aligned for 6 weeks. After this time, the patient noted ment of thyroid ophthalmopathy.
double vision with vertical displacement of the Because of its unique relationship with the infe-
images, especially when looking down. rior oblique, the inferior rectus is prone to destabi-
lization and subsequent slippage after any recession,
Examination but especially after an adjustable recession for a dys-
After the adjustable left inferior rectus reces- thyroid ophthalmopathy. Any of the rectus muscles
sion, this patient had deficient depression of the left can be involved in dysthyroid ophthalmopathy singly
eye resulting in a variable left hypertropia as well as or in combination. The most commonly involved
ptosis of the left lower lid. The left hypertropia is 10 muscle is the inferior rectus followed by medial,
prism diopters in primary position. With this prism superior, and lateral rectus. I have on numerous occa-
held in front of the left eye, single binocular vision is sions weakened both the inferior and the medial rec-
attained, but diplopia reappears when the patient tus for dysthyroid ophthalmopathy.
looks down into the reading position. I have never had an occasion to resect a muscle
in dysthyroid ophthalmopathy because the motility
Diagnosis problem is invariably one of restriction, not weak-
Slipped left inferior rectus after recession for ness. Surgery in this condition should be held off
thyroid ophthalmopathy. until the patients thyroid status has been stabilized.
However, Coats advocates surgery in some active
Treatment/Surgery cases if symptoms demand. I am skeptical of glow-
ing reports of success with adjustable recession of a
Advancement of the slipped left inferior rectus, single inferior rectus, and because of this I warn the
recession of the right inferior rectus, and recession of reader not to be lulled into a complacent attitude
the left superior rectus. Either or both of the reces- when dealing with this very challenging type of stra-
sions may be done with adjustable suture. bismus. In some cases of stable small-angle strabis-
Comment mus and diplopia from thyroid ophthalmopathy,
prism therapy may be the best treatment. In some
On the surface, this seemed like a fairly cases after surgery for a larger restrictive component,
straightforward case where an acquired mechanical it is necessary to use prisms to treat a smaller residual
restriction could be freed in a patient who already vertical or horizontal deviation.
enjoyed good fusion. Unfortunately, in dysthyroid
404
Strabismus case management
Clinical picture
A B
Bilateral thyroid ophthalmopathy involving multiple muscles. A, Fixing OD, looking slightly upward, demonstrating
esotropia and left hypertropia. B, Looking to the right, demonstrating esotropia and right hypotropia.
History Diagnosis
This 62-year-old man has been troubled by Thyroid ophthalmopathy (Graves ophthal-
double vision for the past several years. Images are mopathy) involving multiple muscles, specifically the
separated both vertically and horizontally. In order to right inferior and medial rectus and the left medial
function, he must occlude one eye. He had been treat- rectus.
ed by his internist for a hyperthyroid condition. He is
now stable after medical ablation of the thyroid and is Treatment/Surgery
in satisfactory systemic control treated with thyroid Recession of the medial recti and recession of
supplement. He is also being treated for hypertension the right inferior rectus (one medial rectus and the
and diabetes. inferior rectus muscle could be recessed with an
adjustable suture).
Examination
Visual acuity is OD 20/25 and OS 20/30 while Comment
wearing the correction: OD +1.00+1.75 X 20 It is common for thyroid ophthalmopathy to
degrees, OS +1.00+1.00 X 160 degrees add +2.75. In involve multiple muscles. In this patient, care was
the primary position, the right eye is 20 prism taken to evaluate the relative stiffness of the left infe-
diopters hypodeviated and 15 prism diopters esodevi- rior rectus muscle. Had it shown any restriction at all,
ated. Elevation and abduction of the right eye are it would have been appropriate to recess this muscle
severely limited. The left eye has moderate limitation also. Placing one medial rectus and the inferior rec-
to abduction. Horizontal and vertical diplopia are tus of the right eye on an adjustable suture enables
present to varying degrees in all fields. The separa- fine tuning of the alignment the day after surgery.
tion of images is greatest on attempts to look up and However, the potential problems associated with
to the right. Passive duction testing after anesthetiz- adjustable inferior rectus recession; that is, early or
ing conjunctiva with proparacaine hydrochloride late slippage should always be kept in mind.
reveals severely limited elevation and abduction in
the right eye and moderate limitation to abduction in
the left eye. The remainder of the eye examination is
unremarkable.
405
Chapter 16
Clinical picture
A B C
Right sixth nerve palsy. A, Primary position fixing with the left eye. B, Fixing with the paretic right eye; C, left
gaze; D, right face turn and left gaze enables single binocular vision.
History
This 62-year-old man sustained closed head junctiva is anesthetized with proparacaine hydrochlo-
trauma in a motor vehicle accident 1 year ago. After ride shows no restriction to full abduction of the right
a brief period of unconsciousness, he noted double eye. With extreme right face turn and left gaze, sin-
vision. This has persisted in all fields except in gle binocular vision is achieved and stereoacuity of
extreme left gaze, where he has single binocular 9/9 (40 seconds) is recorded. The remainder of the
vision. In order to drive, watch television, and read eye examination is normal for a person of this age.
with comfort, he must cover one eye. There were no
other injuries from the accident, and this mans health Diagnosis
is otherwise excellent. Traumatic right sixth nerve paralysis.
Examination Treatment/Surgery
Visual acuity with correction is 20/20 in each Full tendon transfer of the superior and inferior
eye. With the left eye fixing, approximately 20 prism rectus muscles of the right eye adjacent to the inser-
diopters of right esotropia is noted. When fixing with tion of the right lateral rectus.
the right eye, the left eye is esodeviated 60 prism
diopters. The right eye cannot abduct to the midline. Comment
Versions are normal in left gaze. The right eye
Unilateral sixth nerve palsy can present in sev-
floats to just short of the midline during a saccade
eral slightly different patterns, and these different pat-
to the right. The velocity of this saccade is approxi-
terns require different types of treatment. In the case
mately one-fourth the speed of the abduction saccade
described, no right lateral rectus function was pres-
of the left eye. Passive duction testing after the con-
406
Strabismus case management
ent. In addition, there was no significant restriction to nerve palsy are bilateral involvement, absence of lat-
passive abduction. This means that a full tendon eral rectus function (paralysis), and mechanical
transfer procedure alone can be effective. On the restriction.
other hand, if restriction of the antagonist right medi- Before doing an extraocular muscle transfer
al rectus had been present, it would have been neces- procedure to treat a paralyzed muscle, the surgeon
sary to deal with this by weakening this muscle. If a should emphasize to the patient that a muscle transfer
recession is done, then an additional full tendon trans- does not restore full ocular rotations. The surgeon
fer of the vertical recti would leave only one anterior should also emphasize to the patient with bilateral
ciliary artery intact (that in the paretic lateral rectus). sixth nerve palsy that diplopia will persist postopera-
This can be done, but it increases the risk of produc- tively even when primary position alignment is
ing anterior segment ischemia, especially in an older achieved because abduction is never full and a sec-
patient. Since the vertical recti have no long posteri- ondary deviation occurs in gaze to the right or left,
or ciliary artery, detachment of these muscles with no creating horizontal and sometimes vertical strabismus
backup arterial blood supply introduces a greater and diplopia. An induced vertical deviation is more
risk of anterior segment ischemia when compared to likely to occur after full tendon transfer. The occur-
detachment of the horizontal recti. rence of vertical strabismus after full tendon transfer
When passive abduction is restricted in a has prompted Rosenbaum et al. to suggest that
patient with sixth nerve palsy, Botox can be injected adjustable sutures be used on the transferred vertical
into the antagonist medial rectus to weaken it either at recti. In any case of strabismus from cranial nerve
the time of surgery or from 7 to 14 days before or palsy, suppression of the second image is advanta-
after surgery. In a case of sixth nerve palsy with suf- geous if fusion is unattainable. In the case of unilat-
ficient lateral rectus function remaining so that the eral sixth nerve palsy I do not recess the sound medi-
paretic eye moves beyond the midline combined with al rectus of the fellow eye because the field of single
only slightly reduced saccadic velocity during abduc- binocular vision can be reduced in the field opposite
tion, a recession of the medial rectus and resection of the paralyzed lateral rectus. Instead of recessing the
the lateral rectus can be effective. In the case of an normal medial rectus a posterior fixation suture may
acute sixth nerve palsy, Botox injection into the be used.
antagonist medial rectus muscle can be effective in A diplopia field plotted with an arc perimeter or
forestalling contracture of this muscle, thereby pro- a bowl perimeter is a useful way to follow the
viding a better chance for effective rehabilitation of progress of sixth nerve palsy, either after surgical
the reinnervated paretic lateral rectus muscle. At least treatment or as it spontaneously resolves. Results can
this makes sense, but the benefit from this prophy- be important for medicolegal reasons. The fields are
lactic use of Botox has not been established. In sur- recorded quickly and easily by determining how
gical treatment of sixth nerve palsy, favorable factors many degrees away from primary position an object
are unilateral involvement, residual lateral rectus can be moved before it is seen doubled. To do this
function (paresis), and absence of mechanical restric- test, the head must be centered in the head support
tion. When a medial rectus recession and lateral rec- (not to the right or left, as is done during visual field
tus resection can be done, postoperative results are testing of each eye) and the head should remain fixed
better than if a muscle transfer is required. while only the eyes move.
Unfavorable factors in surgical treatment of sixth
407
Chapter 16
Clinical picture
A B C
Bilateral sixth nerve palsy greater in the right eye. A, Primary position with left eye fixing. B, dextroversion; C, levoversion
History
This 66-year-old woman sustained closed head because passive adduction of the right eye was mod-
injury in a car accident 14 months earlier. She also erately restricted.
sustained multiple lower limb fractures and uses a
walker. Since the accident, her eyes have crossed and Comment
she is bothered by constant diplopia. Posteratively the patient had anterior segment
ischemia of the right eye characterized by flare and
Examination cell, keratic precipitates, and a dilated fixed pupil.
Visual acuity with correction is OS 20/25-2 and This resolved for the most part after intense topical
OS 20/20-1. Glasses are OD +2.00+0.50 X 180 steroid therapy consisting of 1 drop of 1% pred-
degrees and OS +2.00+0.50 X 10 degrees with a nisolone in the right cul-de-sac every 2 hours while
+2.50 add. Adduction is normal in both eyes. awake. This was reduced gradually over the next 4
Abduction is slightly limited (-1) in the left eye. The weeks to 2 drops a day as the anterior chamber flare
right eye does not abduct even to the midline. and cell reaction subsided. Atrophy of the iris stroma
Approximately 60 prism diopters of right esotropia is persists from the 9 to 12 oclock meridians. The pupil
present in the primary position while fixing with the is also eccentrically dilated to approximately 6 mm
left eye. The esotropia increases when fixing with the with a reduced reaction to light. In addition, during
right eye and in right gaze. The esodeviation is less this period, the patient developed cystoid macular
in left gaze. Saccadic velocity is moderately brisk to edema with visual acuity reduced to 20/200 in the
the left in the left eye. A floating saccade is present right eye. The retinal lesion was not treated. After 6
in attempted abduction in the right eye as the eye weeks, vision improved to 20/30, but the residual pig-
moves from the adducted position to just short of the mentary changes in the macula and a slight increase
midline. in the cataract indicate that visual acuity may not
improve beyond this level. Primary position align-
Diagnosis ment is 10 prism diopters of exotropia, and the patient
Bilateral traumatic sixth nerve palsy more has single binocular vision with a slight left face turn.
severe in the right eye. This case may demonstrate the vulnerability of
an eye in an older patient to anterior segment
Treatment/Surgery ischemia after detachment of the vertical recti. It is
Full tendon transfer shifting the right superior not clear what role the Botox injection in the medial
rectus and right inferior rectus to the right lateral rec- rectus or for that matter the lateral rectus plication
tus was performed. At surgery this patient was noted played in the anterior segment ischemia. However, I
to have a flaccid right lateral rectus muscle. Because have seen two other cases of anterior segment
of this, in addition to the full tendon transfer, a 10 mm ischemia occurring after Botox injection in similar
plication-tuck was done on the right lateral rectus, cases where we thought sufficient anterior ciliary cir-
sparing the anterior artery. Five units of Botox were culation remained.
then injected into the right medial rectus muscle
408
Strabismus case management
Clinical picture
B C
Head posture to achieve single binocular vision after surgery on both eyes. A, Primary position; B, dextroversion;
C, levoversion; D, a chin up left tilt head posture is needed to obtain single binocular vision
History
This 52-year-old woman was involved in a are full. This woman has double vision in all fields of
motor vehicle accident 4 1/2 years ago, sustaining gaze with the images separated horizontally. She is
bilateral sixth nerve palsy. Since sufficient lateral wearing 15 diopters of permanent prism divided 8
rectus function remained, she was treated with reces- prism diopters base out in the right lens and 7 prism
sion of the medial recti and resection of the lateral diopters base out in the left lens. With this prism she
recti for esotropia of each eye, with the surgeries done is able to see objects singly with her head turned to
1 month apart. She now complains of double vision. the left and her chin slightly elevated. If she moves
This is helped some by using base-out prism, by turn- her head even a few degrees or if the object of regard
ing her eyes to the right, or by occluding one eye. moves even slightly she experiences double vision
but can regain single vision fairly easily
Examination
Visual acuity with correction is right eye 20/25
Diagnosis
and left eye 20/30. The patient is wearing the fol- Residual esotropia with diplopia after treatment
lowing myopic correction: right eye 4.25 and left for bilateral sixth nerve palsy.
eye - 3.50 + 0.75 X 35 degrees. Prism and cover test-
ing in the primary position reveals 20 prism diopters Surgery
of esotropia. This decreases to 10 prism diopters Consider re-resection of the left lateral rectus
esotropia in far right gaze and increases to 25 prism muscle and re-recession of the left medial rectus mus-
diopters in far left gaze. No significant A or V pat- cle.
tern or other vertical deviation is noted. There is
moderate limitation of abduction more in the left eye
than the right. Saccadic velocity is brisk to abduction
to either side. Adduction is full and vertical versions
409
Chapter 16
Comment
This patient is happy with the small area of sin- no. If this were present, the two foveas would repel
gle binocular vision and is willing to try to have this each other making any single binocular vision impos-
limited field enlarged by additional surgery. When sible. In this condition the two fovea act as thought
considering a further attempt at gaining a wider range they were similar magnetic poles as they are driven
of single binocular vision several things must be con- apart. Will the relentless secondary deviation of
sidered. First, does the patient have central disruption bilateral sixth nerve palsy keep the eyes from work-
of fusion* The answer is most likely no. If she had ing together? The answer is maybe, but maybe not.
central disruption of fusion she would not be able to If the patient is willing to have realistic expectations,
gain and regain single binocular vision as she does. further surgery is worth a chance.
Does she have horror fusionis? Again the answer is
E F
G H
Pratt-Johnson JA, Tillson G: The loss of fusion in adults with intractable diplopia (central disruption of fusion), Aust NZ J Ophthalmol
16:81-85, 1988.
410
Strabismus case management
Clinical picture
A B
A, Right esotropia when fixing with the left eye. B, In dextroversion the right eye has no abduction
but is esodeviated and hyperdeviated.
History Comment
This 43-year-old woman had an intracranial This patient with acute sixth nerve palsy may
aneurysm clipped 6 weeks earlier. Her right eye recover some or all of her sixth nerve function in the
crossed moderately before the surgery. After surgery, right eye over a period of up to 6 months. To prevent
the right eye crossed completely. The images are so spastic contracture of the unopposed right medial rec-
far apart that they are not very bothersome. tus muscle with additional development of restriction
during the convalescent period, Botox is injected into
Examination this muscle. This patient represents an extreme case
Visual acuity is OD 20/40 and OS 20/20 with- of sixth nerve palsy where the nerve may have been
out correction. Motility testing shows 70 prism transected at surgery. Other milder cases of unilater-
diopters of right esotropia with no right lateral rectus al and bilateral acute sixth nerve palsy can also bene-
function. The remainder of the eye examination is fit from Botox injection. There is no clear-cut evi-
normal. dence that the Botox enables or even hastens recovery
of sixth nerve function, but it does make sense and
Diagnosis there is little downside. The mechanism of action of
Acute right sixth nerve paralysis. Botox in similar cases is to paralyze the medial rectus
muscle for a period of weeks to months. Ptosis of the
Treatment/Surgery upper lid often occurs when the lateral rectus muscle
is injected. To help avoid this, the patient should sit
Botox, 5 units to the right medial rectus muscle. up immediately after injection and should remain
upright for at least 2 hours. The toxin is less likely to
diffuse in the area of the levator palpebri when this
precaution is observed.
411
Chapter 16
Clinical picture
B C
D E
This patient has an acute right third nerve palsy. A, In the primary position, ptosis of the right upper
lid is complete. B, With the right upper lid held up by the right patients finger, the right eye is
exodeviated approximately 40 prism diopters and is slightly hypodeviated. C, Abduction of the right
eye is full. D, The right eye cannot adduct even to the midline. E, On downgaze the right eye
intorts slightly, suggesting unopposed superior oblique function.
History Examination
This 28-year-old man sustained closed head Complete ptosis of the right upper lid is present.
trauma in a motor vehicle accident 13 months earlier. With maximum attempt at elevation using the frontal-
He is concerned because his right eye is closed. is muscle, the right upper lid moves upward about 3
When he raises his right upper lid, he notes that his mm. Forty prism diopters of exotropia and 15 prism
eye is deviated outward, and he sees double. He diopters of right hypotropia are present in the primary
would like to have the right eye straightened and his position. The right pupil is dilated to 6 mm and does
right lid raised. not react to light or accommodative effort. Visual
acuity is OD 20/30 and OS 20/20. This patient under-
stands that if his eye is made straighter and his lid
raised, he will continue to have double vision and that
this double vision may be more bothersome because
the images are closer. In spite of this, he would like
to have surgery to improve alignment of his eyes.
412
Strabismus case management
Diagnosis
Traumatic right third nerve palsy. which is the rule after frontalis lid suspension and
limited upward protective movement (Bell phenome-
Treatment/Surgery non) of the eye. In a patient such as this, who lacks
Maximum recession of the right lateral rectus effective suppression, diplopia can be extremely
10+ mm, right superior oblique tendon transfer with- bothersome. Actually, some of the most agitated and
out trochlea fracture or maximum recession of the distraught patients I have treated have been of this
right lateral rectus and 10+ mm resection of the right category. This problem is especially severe when the
medial rectus with 1/2 to 3/4 muscle width upshift of patient with third nerve palsy is emotionally liable
both muscles. from brain injury. Patients with acquired third nerve
palsy should be counseled thoroughly before surgery,
Comment telling them about the problems associated with post-
The appropriate extraocular procedure, when operative diplopia. In several successfully aligned
successful, can align or nearly align the eye with third cases, it has been necessary to fit the patient with an
nerve palsy, but motility is always limited. When the occluding contact lens or to give glasses with an
lid is raised, postoperatively the involved eye during occluder lens to eliminate the diplopia. On the other
fixation in the primary position is usually slightly hand, if suppression is present for any reason or if
exotropic. Frontalis suspension of the upper lid can vision is poor in one eye alignment can be achieved
be performed at the same time as the extraocular mus- and the patient is pleased. In some cases of complete
cle surgery or it can be done at a second procedure. acquired third nerve palsy it may be best to refrain
Whenever it is done, the ptosis should be undercor- from surgery and simply allow the ptosis to treat
rected to lessen the adverse effect of corneal exposure diplopia.
413
Chapter 16
B A C
A, Primary position alignment with 3 mm ptosis of the left upper lid. B, The left palpebral fissure widens
on right gaze. C, The left palpebral fissure narrows on left gaze. D, The left eye does not elevate. E,
The left eye does not depress and the left upper lid retracts from aberrant regeneration during attempted
downgaze.
414
Strabismus case management
History Diagnosis
This 36-year-old woman was involved in a Traumatic third nerve palsy with aberrant
motor vehicle accident 2 years earlier. She was com- regeneration after recess-resect of the horizontal recti
atose for 14 days. When she regained consciousness, with upshift.
she had constant double vision. She is also bothered
by generalized left-side weakness. She has difficulty Treatment/Surgery
walking, has slurred speech, cries easily, and has dif- Recession of the left lateral rectus 9 mm, resec-
ficulty with memory. A left lateral rectus recession of tion of the left medial rectus 10 mm with upshift of
9 mm and a left medial rectus resection of 10 mm the insertion of both horizontal recti one half muscle
with one-half muscle width upshift was done 4 width.
months ago.
Comment
Examination
In cases of unilateral palsy of the third nerve
The patient has visual acuity of OD 20/30 and with aberrant regeneration, such as this, a
OS 20/20. The 30 prism diopters of left exotropia recession/resection procedure with upshift of both
and 15 prism diopters of left hypotropia that had been muscles can be effective in straightening the eyes in
present in the primary position before recent surgery the primary position. There is no effective way to
has been nearly eliminated, leaving 5 prism diopters deal surgically with the aberrant regeneration. Since
of exotropia resulting in satisfactory appearance. the ptosis in this patient is only moderate, possibly a
The left pupil is dilated to 6 mm and is nonreactive to result of the effects of the aberrant regeneration, no
light or accommodation. The left eye has nearly full treatment is required. Patients with third nerve palsy
adduction but neither elevates nor depresses more will always see double in nearly every direction
than a few degrees. During dextroversion of the left unless they are successful in suppressing one image,
eye, the left upper lid elevates. This lid is photic on usually from the paretic eye. Aberrant regeneration
gaze to the left. On attempted downgaze, the left eye occurs in approximately two-thirds of patients with
remains near the primary position but the left upper third nerve palsy, congenital or traumatic.
lid retracts. The remainder of the eye examination is
unremarkable.
415
Chapter 16
Comment
After the first surgical procedure, the childs
eyes were aligned horizontally but he persisted with a
large right hypotropia. After the second procedure,
the patient has only a small right hypotropia or left
hypertropia and slight ptosis of the right upper lid.
This patient was treated initially with a recess/resect
Congenital right third nerve palsy aligned surgically . A procedure of the right eye with upshift because
small residual right exotropia and ptosis of the right upper adduction was only moderately limited. There was
lid remain. no limitation to passive ductions in either eye.
Congenital third nerve palsy has many expres-
sions. This patient had fairly mild congenital third
nerve palsy that was treatable with a recession/resec-
History tion and did not require a muscle transfer procedure.
This 14-month-old boy was brought by his par- Since the ptosis in this case is mild, no treatment is
ents for examination because his right eye deviated indicated now. Before school-age, it may be appro-
outward and downward. This had been present and priate to do a small right levator resection.
unchanging since birth. They also thought the right Free alternation in this patient rules out ambly-
upper lid drooped. The child is otherwise healthy opia. I have treated several infants with congenital
and is developing normally, with all milestones third nerve palsy who preferred fixation with the
reached on time or early. paretic eye because vision was better in this eye. If
vision is equal in patients with congenital third nerve
Examination palsy, they frequently alternate fixation having a
This patient fixed and followed well with either large secondary deviation when fixing with the paret-
eye. While fixing with the left eye, the right eye was ic eye. The potential for amblyopia in the patients
down and out and a mild right ptosis was present. with congenital third nerve palsy should not be
When fixing with the right eye, a large left hyper- ignored while focusing on the strabismus alone. If
tropia with exotropia was present. With the left eye fixation preference is noted and the non-preferred eye
fixing, the right eye was 20 prism diopters exotropic appears normal, occlusion therapy should be carried
and 15 prism diopters hypotropic. With the right eye out. However, it should be closely monitored. I saw
fixing, the left eye was 30 prism diopters exotropic a patient with third nerve palsy who developed
and 25 prism diopters hypertropic. Levator function intractable occlusion amblyopia after several weeks
in the right eye was only mildly limited. The right of full-time occlusion at six months of age. In addi-
pupil responded normally. The remainder of the eye tion to the amblyopia, a grotesque secondary devia-
examination was normal. tion was created by fixing with the paretic eye. I
believe that imaging studies with CT scan or MRI
Diagnosis should be done in all cases of congenital third nerve
Congenital incomplete right third nerve palsy. palsy to rule out structural brain abnormalities.
416
Strabismus case management
Clinical picture
History Comment
This 6-month-old patient had both eyes Severe congenital third nerve palsy requires
markedly deviating outward and bilateral ptosis. In more than a recession/resection procedure because
order to see, the child habitually used her left forefin- adduction is absent. In this case a superior oblique
ger to elevate the left upper lid. tendon transfer with fracture of the trochlea was done.
Recession of the lateral rectus provides additional
Examination help toward centering the eye. The ptosis procedure
Both eyes are deviated downward and outward. done later should aim at undercorrection because the
There is little movement toward elevation or adduc- cornea is at risk for exposure. A review of our
tion. Both lids are photic and there is no detectable patients revealed that approximately two-thirds of
levator function. Both pupils are mid-dilated and children with congenital third nerve palsy have some
react sluggishly to light. The infant appears to have evidence of aberrant regeneration, suggesting that
moderate psychomotor retardation. The role of the trauma to the nerve has occurred. Patients with con-
visual deficit in causing this delay cannot be deter- genital third nerve palsy do not have diplopia because
mined fully at this time. The eyes are otherwise nor- of effective suppression. This patient is one of the
mal. very few who in my experience had successful frac-
ture of the trochlea. I have abandoned this technique
Diagnosis because of difficulty with the fracture and/or unin-
tended transection of the tendon making transfer
Bilateral complete congenital third nerve palsy.
impossible, especially in older patients.
Treatment/Surgery
First procedure (age 8 months): recession of
both lateral recti, bilateral superior oblique tendon
transfer with trochlear fracture. Second procedure
(age 10 months): bilateral (temporary) frontalis sus-
pension of the upper lids using heavy nylon suture.
417
Chapter 16
Clinical picture
A B C
A, Left exotropia; B, limited adduction in the left eye; C, normal adduction in the right eye.
History Comment
This 26-year-old man sustained an injury to the This patient with a long-standing left sensory
left eye at age 7 when he was struck with a nail. The exotropia is bothered socially and in business deal-
corneal laceration was repaired and the damaged lens ings by a feeling that he describes as people dont
removed. No optical or other treatment was given to know where I am looking. Justification for treat-
the eye. His left eye has gradually drifted outward. ment of this patient may be under the heading all
He would now like to have his eyes straightened in humans have the right to look like a human. The
order to have improved eye contact. He feels uncom- only normal human ocular alignment is to have
fortable talking to people, and he states that he is not orthotropic or aligned eyes. Therefore, straighten-
sure if people know where he is looking when he is ing the left eye in this patient with visual acuity of
talking to them or trying to get their attention. counts fingers in that eye is a functional procedure.
Patients like this are extremely grateful for any
Examination improvement in their appearance. Many patients with
Visual acuity in the right eye is 20/20 without large-angle exotropia deny that a problem exists and
correction, and in the left eye it is counts fingers at are reluctant to seek help. Compared to those with a
3 feet not improved with lenses. Fifty prism diopters similar size esodeviation, the patient with large-angle
of left exotropia is present in the primary position exotropia is typically more willing to endure strabis-
measured with the prism and light reflex test mus and not seek treatment or will seek it later.
(Krimsky). Ductions of the right eye are normal. Several character actors have actually capitalized on
Adduction in the left eye is limited slightly at -1. a large angle exotropia to create a sinister or devious
During extremes of upgaze and downgaze, the image. On the other hand, an esotropia imparts a
exotropia increases to 70 prism diopters, creating an foolish image and an affected patient is more likely
X pattern. to seek treatment. Any adult patient with manifest
exotropia who also retains vision in the deviated eye
Diagnosis has an enlarged field of peripheral binocular vision.
Sensory left exotropia with X pattern. These patients should be warned that they will have a
decrease in their binocular field of vision after the
Treatment/Surgery eyes are straightened. This can be disturbing to
patients at first. They often report a sensation of hav-
Recession of left lateral rectus 9 mm, resection ing tunnel vision after surgery. This sensation
of left medial rectus 8 mm. always goes away, with the result that patients report
normal vision in weeks or months.
418
Strabismus case management
Clinical picture
B A C
History Comment
This 43-year-old woman had eye muscle sur- This patient is typical of many patients who
gery on the left eye at age 5 for esotropia that had have had eye muscle surgery done in the 1950s. She
begun during the first year of life. Vision has been had a transconjunctival incision over the insertion of
very poor in the left eye because of what the patient the left medial rectus. As often occurs when this inci-
described as a hole in the retina. The patient teach- sion is used, the conjuctiva is scarred with a rough-
es fourth grade and complains that her students do ened, red mass over the entire medial conjunctiva. At
not know where she is looking. She would like to surgery the medial rectus was found 11.0 mm from
have her eyes straightened. the limbus. Because the conjunctiva was rough and
red, it was excised to the plica, which had been pulled
Examination closer to the nasal limbus by scarring from the previ-
Visual acuity with correction is OD 20/20 and ous surgery. The plica was sutured down to sclera
OS counts fingers at 4 feet. Her refraction is OD - and resection of the left lateral rectus was done.
6.50+2.75 X 75 degrees and OS -3.75+2.75 X 35 It is a good idea to slightly undercorrect
degrees. In the primary position, the left esotropia patients like this with esotropia. Postoperatively, they
measures 30 prism diopters. There is also 5 prism would be more likely to notice and be dissatisfied
diopters of left hypotropia. Abduction is moderately with 5 prism diopters of exotropia than 5 prism
limited in the left eye and a roughened, raised red diopters of residual esotropia. This woman has
conjunctival scar is noted medially in the left eye. An become accustomed to (if not happy with) the esode-
inactive chorioretinal scar involving the macula of viation and will be very grateful for a significant
the left eye accounts for the poor vision in that eye. reduction but may be unhappy with even a small
overcorrection. Justification for straightening a
Diagnosis deeply amblyopic eye such as this are (1) normaliza-
Residual sensory esotropia OS, with conjuncti- tion of the oculofacial relationship with improved
val scar. interpersonal relations and (2) increase in the periph-
eral binocular field.
Treatment/Surgery Coats and Paysse have shown that applicants
who have had digitally altered photos showing stra-
Exploration of the left medial rectus, with pli- bismus receive lower evaluations on applications
caplasty and re-recession of the left medial rectus if compared to when their pictures are unaltered and
possible or marginal myotomy of a fully recessed left show straight eyes. This demonstrated that strabis-
medial rectus and resection of the left lateral rectus. mus can be a handicap when it comes to making a
The amount of surgery is determined at the time of favorable impression.
surgery. One or both of the muscles may be placed
on an adjustable suture.
419
Chapter 16
A B
A, Right eye fixation showing 10 prism diopters of left hypotropia. B, Left eye fixation with 15
prism diopters of right hypertropia.
History Diagnosis
This 21-year-old woman had eye muscle sur- Residual esotropia, asymmetric DVD with
gery done on both eyes in Germany between ages 3 small true left hypotropia and falling left eye.
and 5 years. Since that time, she has had some cross-
ing of her eyes. More recently, she observed that the Surgery
left eye appears to be lower than the right most of the Recession of right superior rectus 7 mm, re-
time. This patient wishes to have the alignment recession of the left medial rectus.
improved by surgery if it is possible.
Comment
Examination
This patient demonstrates the combination of
Visual acuity with correction is OD 20/20 and true right hypertropia (left hypotropia) along with dis-
OS 20/30. The patient wears contact lenses with the sociated vertical deviation. The left medial rectus
following correction: OD -6.50, OS -7.00. She muscle was selected for re-recession because there
prefers to fix with the right eye. While fixing with the was a slight limitation of passive abduction in this eye
right eye in the primary position, the left eye is esode- noted at surgery. The left medial rectus was found
viated 15 prism diopters and is approximately 10 approximately 9 mm from the limbus. It was re-
prism diopters hypotropic. When the nonfixing left recessed to 11.5 mm from the limbus and the scarred
eye is occluded, it drifts upward above the midline overlying conjunctiva was recessed approximately 5
approximately 10 prism diopters with an excycloduc- mm. The right superior rectus was recessed 7 mm,
tion. This left eye sursumduction and excycloduction which is more than would be done ordinarily for 15
movement is slow and vergence-like, making it a prism diopters of hypotropia. This larger recession
DVD-type response. When the cover is removed was done because of the additional DVD response.
from the left eye, the eye drifts down (deorsumduc- In cases of falling eye with poor vision in the
tion) to 10 prism diopters of hypodeviation while the hypodeviated eye, it is more appropriate to recess the
right maintains fixation. When the left eye takes up inferior rectus of the hypodeviated eye. However, in
fixation but the right remains uncovered, the right eye this patient, the superior rectus recession made more
assumes 15 prism diopters of hypertropia. While fix- sense despite the fact that this eye was habitually used
ation continues with the left eye and the right eye is for fixation, because a larger recession can be done
occluded, it moves up 10 prism diopters more with a safely on the superior rectus in contrast to the inferi-
slow vergence movement and excycloduction. No or rectus without correction for lid fissure changes.
latent nystagmus is noted. Ductions are full in both
eyes. The remainder of the eye examination is unre-
markable.
420
Strabismus case management
This 46-year-old man has poor vision in the left eye that over a period of
many years has become hypodeviated. The hypodeviated eye has a pulsat-
ing vertical nystagmoid movement. When the nonfixing hypodeviated left eye
is occluded, it undergoes a sursumduction in a DVD-type response, moving
several prism diopters above the midline. When the occluder is removed, the
eye returns to a position below the midline. This patient is more typical of the
falling eye syndrome, which has also been called the Heimann-
Bielschowsky phenomenon. In this case, the inferior rectus muscle was
recessed 5 mm, resulting in improvement in the primary position alignment.
421
Chapter 16
Clinical picture
History
This 9-year-old girl has been noted by her par- cases, without elevation of the eye on forced closure
ent to have a droopy right upper lid and a right eye of the lid, it is necessary to determine if there is a
that is chronically deviated downward. This has been restriction to passive elevation. When restriction is
present since birth. Her health is otherwise normal. encountered it must be freed before further surgery is
done to recess or resect the vertical recti or transfer
Examination the horizontal muscles. In the case described here,
Visual acuity is 20/20 in each eye. Cycloplegic since there was no restriction to passive elevation a
refraction in both eyes is +0.75. In the primary posi- full tendon transfer was done. In other cases with
tion, 20 prism diopters of right hypotropia is meas- similar clinical characteristics but that differ slightly
ured. On maximum attempt at looking up, the left eye in that the eye can elevate well above the midline,
reaches just a few degrees short of the midline. With along with evidence of vertical rectus function, a ver-
forced lid closure, the eye moves up only a few tical rectus recession/resection procedure can be
degrees more but is well short of full elevation, indi- done.
cating a weak Bell phenomenon. In some cases, a full tendon transfer will pro-
duce a new horizontal strabismus. When this occurs,
Diagnosis it is usually an exodeviation. The method that has
Double elevator palsy of right eye. been suggested to avoid this complication is use of
an adjustable suture, but young children in need of
Treatment/Surgery surgical treatment for double elevator palsy are not
ordinarily suited for an adjustable suture procedure
Full tendon transfer of the right lateral and right
on a transferred muscle. In cases of double elevator
medial recti to a point adjacent to the insertion of the
palsy with postoperative horizontal strabismus, the
right superior rectus (after confirming free passive
vertically transposed recti can be recessed or resect-
elevation of the right eye).
ed appropriately or the horizontal recti of the fellow
Comment eye can be recessed/resected or both. Children with
double elevator palsy may have excellent stereoacu-
Double elevator palsy is a relatively rare and ity in downgaze. This can be compromised after the
enigmatic strabismus. In some cases, the involved eyes are treated surgically. Double elevator palsy in
eye can elevate fully with forced lid closure (Bell its several forms is a difficult and frustrating, but for-
phenomenon). At other times, such as in this case, the tunately rare, type of strabismus to manage surgical-
eye does not elevate. In cases with intact Bells phe- ly.
nomenon, a supranuclear palsy is confirmed. In other
422
Strabismus case management
Clinical picture
A B C
A, Left hypertropia fixing with the right eye; B, The right eye elevates only a few degrees above the midline; C,
Both eyes depress fully.
History
This 22-year-old had repair of a blowout frac- which usually occurs about a week after the injury.
ture and associated facial trauma that resulted from This fracture repair is done with direct visualization
injury incurred in a motor vehicle accident 8 months of the orbital floor. Exposure is obtained through a
ago. Since the surgery, the right eye on the side of subciliary lid skin incision or by means of an incision
repair has been down or at times the left eye has behind the lid through the inferior fornix. The pro-
been up. The patient reports that he sees everything lapsed orbital contents are then teased out of the
double except when he looks in far downgaze. bony defect and the defect in the orbital floor is cov-
ered with a splint made of thin plastic material.
Examination Unfortunately this is not always the end of the story.
Visual acuity is 20/20 in each eye. Prism and Even if all of the incarcerated tissue has been
cover testing shows 14 prism diopters of right removed from the fracture, trauma to soft tissue--
hypotropia when fixing with the left eye and 20 prism including the inferior rectus and surrounding fascia
diopters of left hypertropia fixing with the right eye. and fat can cause restrictions that limit elevation and
Elevation of the right eye is limited to only a few produce a hypotropia of that eye, resulting in diplop-
degrees above the midline, even with maximum ia. In some cases the inferior rectus sustains nerve
effort. Numbness of the right infraorbital area is pres- damage. In this case, after repair either a hypertropia
ent. In downgaze the stereo fly can be fused (gross results or a persistent restrictive hypotropia is present
stereopsis 3000 seconds). The remainder of the eye masking a paretic inferior rectus, that may be recog-
examination is normal. nized only after restriction to elevation has been
freed. When this is accomplished in such a case, lim-
Diagnosis itation in both upgaze and downgaze can result.
Right inferior rectus restriction after blowout A wide variety of traumatic strabismus entities
fracture of the orbit. can result from direct trauma to the muscles, orbital
fascia, and bones around the orbit. In this type of
Treatment/Surgery case, the motility repair can be complicated and there-
fore must be planned and executed based on the
Inferior rectus recession of right eye using
unique motility findings. Recession, resection, and
adjustable suture.
transfer are dictated by the residual function of the
Comment muscles and use of these procedures depends on both
innervational and mechanical factors. In most cases
As with any type of strabismus after trauma, of traumatic motility disturbance that I have treated, it
blowout fracture presents a complex and varied clini- has been possible at best to find a limited area of com-
cal picture. The acute stage of blowout fracture with fortable single binocular vision with residual areas of
prolapse of orbital contents into the maxillary sinus is diplopia.
usually repaired after the swelling has subsided,
423
Chapter 16
Clinical picture
A B C
Acute blowout fracture of the right orbit. A, Primary position; B, limited downgaze in right eye
(reverse leash effect); C, severely limited upgaze of the right eye (leash effect). D, Coronal
CT shows defect in right orbital floor with orbital contents prolapsed into the maxillary sinus.
History Comment
This 6-year-old boy was struck in the right eye Acute blowout fracture of the orbit wall (usual-
by the heel of a playmates shoe while wrestling at ly the floor) is now treated in most cases by the ocu-
play 6 days ago. The right eye was moderately loplastic surgeon. The surgical approach to the
swollen immediately after the injury. The child saw orbital floor is through a subciliary incision made in
double after he was struck, and he continues to see the skin of the lower lid or through an inferior fornix
double at all times. incision. With either incision, inferior orbital rim
periosteum is incised below the inferior orbital sep-
Examination tum, and the periosteum is elevated to expose the
Visual acuity is OD 20/30 and OS 20/20. No orbital floor defect. Prolapsed orbital contents are
significant refractive error is present. The eyes are carefully extracted from the maxillary sinus, and a
straight in the primary position and 6/9 stereo dots thin plastic sheet, either preformed or cut and shaped
(80 seconds) are seen. Depression of the right eye is to size, is placed over the defect. Unfortunately,
moderately limited and elevation of this eye is severe- adhesions in and around the orbital soft tissue, includ-
ly limited. There is numbness over the medial aspect ing the inferior rectus, can cause restricted eye move-
of the right inferior orbital rim. CT scan of the orbits ment even when freeing of the prolapsed material has
shows a bony defect of the right orbital floor with been complete. If motility continues to be limited
prolapse into the maxillary sinus of the orbital con- after surgical repair of a blowout fracture, appropriate
tents, possibly including the right inferior rectus mus- eye muscle surgery, usually inferior rectus recession,
cle. on the involved side can be carried out. However, if
paresis of the inferior rectus is present, freeing of
Diagnosis restriction to elevation must be followed by an inferi-
Acute blowout fracture of right orbital floor. or rectus resection, or, if the inferior rectus is non-
functioning, muscle transfer must be done by shifting
Treatment/Surgery the horizontal recti to the inferior rectus insertion.
Removal of orbital contents from the maxillary
sinus and repair of the fracture defect with a splint.
424
Strabismus case management
Clinical picture
B A C
A, This patient has bilateral ptosis, chin elevation, and gaze downward with exotropia;
B, The patients sister; C, The patients mother has only the right eye affected
History Comment
This 16-year-old girl has had chin elevation Congenital fibrosis syndrome is inherited as
and exotropia in downgaze all of her life. She also autosomal dominant with nearly complete pene-
complains that both her upper lids droop. Her sister, trance, although involvement may vary. The inferior
mother, maternal uncle, and maternal grandfather are and medial rectus muscles are often thin tight bands
also affected. in this condition. The medial rectus muscle also
tends to course upward to the insertion, suggesting an
Examination origin in the orbit lower than is usually seen. Results
The most striking feature of this patient (and all of surgery for fibrosis syndrome are frequently disap-
other similarly affected patients) is the chin-up posi- pointing because of residual restriction to elevation
tion with bilateral ptosis. No levator function is pres- and continued convergence on upgaze. However,
ent. On attempted upgaze (actually attempting to lift after surgery some patients are able to assume a near-
the eyes to the primary position), the frontalis is used ly normal head posture. Treatment of congenital
to raise the lids, but with this effort only a few mil- fibrosis syndrome, though difficult and not producing
limeters of lid elevation is accomplished. Also, when excellent results, is certainly worth doing. Most
the patient attempts to look up, the eyes converge. patients appreciate any improvement, even though
Visual acuity is 20/30 in each eye. minimal. The degree of involvement varies from
patient to patient. In general the less severe the fibro-
Diagnosis sis, the better the result from surgery.
Congenital fibrosis syndrome.
Treatment/Surgery
Bilateral inferior rectus recession with frontalis
suspension of the upper lids done at the same proce-
dure with the extraocular muscles or at a second pro-
cedure. If esotropia is a problem the medial recti can
be recessed
425
Chapter 16
Clinical picture
A B
History Diagnosis
This 6-year-old girl has a blank look and both Mbius syndrome.
eyes are crossed. Shortly after birth and throughout
infancy she had some difficulty feeding. Now she is Treatment/Surgery
healthy and eats without difficulty. Bimedial rectus recession to 10.5 mm.
Examination Comment
Simply observing this child provides sufficient Children with Mbius syndrome or bilateral
information to make a diagnosis of Mbius syn- congenital paresis of the sixth and seventh cranial
drome. The eyes are moderately crossed, and the nerves present a typical clinical picture, as demon-
nasolabial fold is absent bilaterally. This gives a dull strated by this child. Surgery consisting of bimedial
facial expression. Neither eye can abduct beyond the rectus recession can at best achieve alignment in the
midline. When adduction is attempted with either primary position. Postoperatively, abduction will not
eye, there is a convergence response in the fellow eye. be restored. Parents must be warned before surgery
The eyes elevate and depress normally. Visual acuity that they should have limited and realistic expecta-
is 20/30 in each eye. Cycloplegic refraction is OD tions about results. I have not done muscle transfer
+1.00+0.50 X 30 degrees, OS +1.00 sphere. The dis- procedures in these patients. Diplopia is not a trou-
tal third of the tongue appears to be atrophic (looked blesome symptom in such patients because the stra-
for specifically because this is a typical feature of bismus is present from birth with suppression. In my
Mbius syndrome). experience Mbius syndrome is a true congenital
esotropia along with Duane I, and both also happen to
be conatal.
426
Strabismus case management
Treatment/Surgery
3.0 mm right superior rectus recession.
Comment
Vertical diplopia occurring suddenly in an older
person, as it did in this case, is usually caused by
fourth nerve palsy, presumably a microvascular
insult. In a slightly younger patient it may result from
thyroid ophthalmopathy. However, there appears to
be another cause in this patient. The key to the dif-
ferent etiology is that this is a comitant vertical devi-
This patient is shown 1 day after 3 mm recession of the
right superior rectus. Except for slight reaction around the ation. An acquired comitant vertical strabismus is
right superior rectus (and right upper lid), the patient looked called skew deviation. It is believed that this is a
the same before surgery. supranuclear motility disturbance caused by minor
brain stem insult, usually a microvascular accident.
Prism therapy is adequate for most patients like this.
History However, if the patient is not happy with prism for
any reason, a surgical procedure involving recession
This 81-year-old man complained of vertical
of a single vertical rectus muscle can be successful.
diplopia that has bothered him for 3 years. He is oth-
Skew deviation; that is, without a paretic muscle is
erwise healthy for a man of his age. Images are sep-
usually seen in older patients, is supranuclear, and has
arated vertically by about the height of his television
a presumed vascular cause that is not usually identi-
screen. He had a slight stroke 3 years ago. This has
fied specifically.
left him with no apparent residual problem other than
Some strabismologists discourage use of the
double vision.
term skew because it might presume etiology but not
Examination describe the strabismus. In place of the term skew
deviation it may be more useful to say that this
Visual acuity is OD 20/25-1 and OS 20/40+1 patient has an acquired comitant right hypertropia.
with correction of OD -1.50+0.50 X 80 degrees and The presumed supranuclear microvascular cause is
OS -1.75+0.75 X 100 degrees and a +2.75 add. There inferred. In my opinion, this type of strabismus does
is 4 diopters of base-down prism in the right lens and not require extensive workup. Evaluation of blood
3 diopters of base-up prism in the left lens. Prism and pressure and blood sugar is indicated, but further
cover testing with the patients distance correction in evaluation should be based on the patients other
a trial frame shows a comitant 10 prism diopter right health considerations. Patients like this are often sub-
hypertropia. This vertical deviation does not change jected to needless, expensive imaging studies before
in right or left head tilt. No torsion is measured with they are seen by an ophthalmologist.
the double Maddox rod. The remainder of the eye
examination is unremarkable except for incipient
cataracts slightly greater in the left eye. The patient
is unhappy with wearing prism because he can see
well at near without glasses, but he has diplopia when
he takes them off to read the newspaper. He would
like to have eye muscle surgery if this would allow
him to get rid of his need for prism.
427
Chapter 16
Clinical picture
A B
A, Left esotropia (30 diopters) without glasses. B, Right esotropia (20 diopters) with glasses.
History Diagnosis
The 4-year-old boy was examined and treated Acquired esotropia, partially accommodative,
initially by an optometrist at age 2 years. Six months increasing with prism adaptation in a prism adapta-
before, at age 1 1/2 years, his parents noted that his tion responder.
eyes were turning in. This in-turning was intermittent
at first, but after a few months it was constant. At his Treatment/Surgery
initial examination the child was given glasses to cor- Bimedial rectus recession to 10 mm from the
rect farsightedness and to help straighten his eyes. He limbus for 35 prism diopters of residual esotropia.
has worn these glasses faithfully for 2 years. Several
interim checkups indicated no need to change the pre- Comment
scription of the glasses but the esotropia remained.
Acquired esotropia, which frequently has a
The child was referred for further evaluation and pos-
refractive component, presents a clinical challenge in
sible treatment.
that the proper amount of surgery can be difficult to
Examination determine. Surgery done for the residual angle while
wearing full hyperopic correction could result in
The patient is able to fix and follow with either undercorrection. To avoid this, the prism adaptation
eye, takes up fixation freely with either eye, and test may be used to uncover a patients true or maxi-
appears to be at ease wearing his glasses, which mum esodeviation. For this test, Fresnel prism of suf-
measure OD +2.00, OS +2.50. With these, he is able ficient strength to fully correct residual esotropia is
to see 20/40 in each eye tested with pictures. His placed on the patients glasses. After a period of
esotropia with glasses measures 20 diopters at dis- adaptation, which may be hours, days, or weeks, the
tance and at near. Ductions are full and no A or V deviation is re-measured.
pattern is present. The esotropia without correction In some cases, the initially placed prisms con-
increases to 35 diopters at distance and 30 diopters at tinue to fully correct the angle. In this case, the
near. Cycloplegic refraction is OD +2.00 and OS patient is said to not respond to the prism adaptation.
+2.50, indicating no need to change prescription in If Worth four light fusion is measured, surgery is done
the glasses. To perform prism adaptation, fully cor- in an appropriate full amount for the residual angle;
recting base-out Fresnel prisms (20 prism diopters) that is, the amount corrected by the prism. In other
were placed on his spectacles. After 2 weeks, 15 cases, the angle increases or builds after the initial
prism diopters of esotropia was measured while the full correction with prism, and more prism must be
Fresnel prisms were in place. After wearing 35 added to neutralize the esotropia. Such patients are
diopters of base out prism for another two weeks, said to respond. More surgery is indicated for
cover testing showed 2 prism diopters of esotropia patients who increase their angle of deviation after
and the patient fused the Worth four lights at near. wearing prisms that initially fully correct the devia-
This indicated a total deviation of 35 prism diopters tion.
of esotropia while wearing full hyperopic correction
in this prism adaptation responder.
428
Strabismus case management
For practical purposes, a maximum of 60 prism for the initially adapted angle; that is, a bimedial
diopters of Fresnel prisms is used for the prism adap- recession 10.0 mm from the limbus for 35 prism
tation test. If more esotropia builds, no more prism is diopters of esotropia. In the study protocol of the
added and maximum esotropia surgery is done. In prism adaptation test, prism would have been added if
the case presented here, a large amount of surgery the patients angle continued to build up to a maxi-
was done for what appeared to be only a 20 prism mum of 60 prism diopters before discontinuing adap-
diopter residual esotropia. Because the esotropia tation. I believe that sufficient information can be
increased to at least 35 prism diopters after adapta- gleaned in most cases from one session of adaptation.
tion, more surgery was justified. This patient will Those patients who adapt by increasing their
wear his glasses after surgery as long as they are nec- esotropia while wearing fully correcting prisms
essary to maintain alignment. should have an appropriate amount of surgery, but it
We could have elected to do surgery in this is probably impractical to attempt to titrate the
case after one session of prism adaptation, selecting amount too finely.
an amount of bimedial rectus recession appropriate
A B
A, Another typical acquired esotropia; B, residual esotropia while wearing fully correct-
ing plus lenses. She is a candidate for prism adaptation. With prism adaptation, sur-
gery is done either for the residual angle (as shown in B) or for the adapted angle,
which means that more surgery will be done if the esotropia increases while wearing
prism that corrects the deviation initially.
429
Chapter 16
Clinical picture
A B
A 47-year-old woman with limited motility and ptosis. A, Primary position B, upgaze.
History Treatment/Surgery
For more than 20 years, this 47-year-old Recession of the right superior rectus with
woman has had diplopia associated with a known appropriate prism postoperatively for residual devia-
diagnosis of chronic progressive external ophthalmo- tion; bilateral frontalis suspension aimed at some
plegia (CPEO). She has no other systemic disease undercorrection.
and specifically has no heart problems indicative of
Kerns-Sayre syndrome which includes CPEO, pig- Comment
mentary retinopathy, and complete heart block. She Patients such as this pose a challenge to the
is annoyed by constant double vision and ptosis. She strabismus surgeon. Obviously, eye muscle surgery
has chronic exposure of the right cornea that requires will not restore normal or even near normal move-
frequent instillation of topical lubricating drops. She ment. Prism therapy is appropriate in some cases, but
would like to have treatment that would enable some in others, the deviation is so great that prisms are
single binocular vision, even if it were only a small heavy, cumbersome, and therefore impractical. In
area. that case, appropriate recessions and resections of the
rectus muscles can be carried out in an attempt to
Examination align the eyes at least in the primary position. A
Visual acuity is OD 20/40 and OS 20/30 with smaller residual deviation, either horizontal or verti-
correction as follows: OD -2.00+1.75 X 90 degrees cal (or both), can then be treated with prism. It is also
and OS -3.25+2.00 X 100 degrees. Ocular motility is frequently necessary to treat these patients with tem-
severely limited in all fields. The patient can move porary Fresnel prism because the deviation tends to
her eyes only slightly away from the primary position be variable. Because of the possibility of Kern-Sayre
in any direction. In primary gaze, she has vertical syndrome, CPEO, retinitis pigmentosa, and heart
diplopia. Images are fused after placing a 15 diopters block, all patients with CPEO should have an electro-
base-down prism in front of the right eye. The cardiogram and a careful retinal examination, includ-
remainder of the eye examination is unremarkable. ing in some cases an electroretinogram. As a final
resort, a patch or an occluding lens may be used to
Diagnosis eliminate diplopia.
Chronic progressive external ophthalmoplegia
(CPEO).
430
Strabismus case management
Clinical picture
A B
A, Ptosis of the left upper lid. B, Left hypertropia apparent when the lid is lifted.
History Treatment
This 46-year-old has a 10-year history of recur- While Mestinon can be very effective for treat-
ring visual complaints secondary to ocular myasthe- ment of the systemic manifestations of myasthenia
nia gravis. These are characterized by episodes of gravis, this medication is not very useful for the treat-
horizontal and vertical diplopia and by ptosis. He is ment of diplopia. Instead, prednisone taken in doses
being treated with Mestinon under the supervision of of 10 to 80 mg every other day can help eliminate or
a neurologist. He is also using oral prednisone inter- reduce the double vision. Since the diplopia from
mittently in doses up to 80 mg every other day, with ocular myasthenia is variable and responsive to treat-
the dosage titrated depending on his visual symptoms. ment with oral corticosteroids, surgery is rarely indi-
cated. However, in a few cases where the diplopia
Examination has been long-standing and refractory to steroid treat-
Visual acuity is 20/20 in each eye with the fol- ment, we have done surgery. If, for example, the left
lowing correction: OD -1.00 and OS -0.75. At the hypotropia in this man remained for several months
time the above photograph was taken the patient was in spite of maximum steroid dosage, we would con-
obviously having no difficulty with diplopia because sider doing strabismus surgery. In this patient I
of the complete ptosis of the left upper lid. However, would do a left superior rectus resection or possibly,
2 months later, the ptosis resolved completely and 15 but less likely, a left inferior rectus recession. For a
prism diopters of left hypotropia remained. The devi- deviation larger than 15 diopters, a recession of the
ation was nearly comitant at this time, and there was right superior rectus could be added. In cases like
a slight limitation of elevation of the left eye. this, recession is actually an attractive choice because
the procedure is tissue sparing and potentially
Diagnosis reversible. When contemplating surgery in a case of
Myasthenia gravis with ocular manifestations. ocular myasthenia gravis such as this, it is necessary
to weigh all of the variables and to be sure that the
specific needs of the patient are kept at the forefront.
If the surgery must be undone it is always easier
and more effective in my hands to advance a previ-
ously recessed muscle than to recess a previous
resected muscle.
431
Chapter 16
432
Strabismus case management
Clinical picture
A, Left hypertropia in a patient with traumatic disinsertion of the left inferior rectus; B, early postoperative normal
depression of left eye after reattachment of a traumatically disinserted left inferior rectus muscle; C, normal
appearance several months after surgery.
History Examination
This 16-year-old girl hit her face on a screen Visual acuity is corrected to 20/20 in each eye
door 4 months earlier. Immediately afterward she with contact lenses: OD -2.50 sphere, OS -3.00
noted double vision, with images being vertically sep- sphere. In primary position, 16 prism diopters of left
arated. The double vision was worse in downgaze. hypertropia is present with the right eye fixing. A
She stated that she saw a single image in upgaze. A large right hypotropia measuring 30 prism diopters is
CT scan of the orbit done elsewhere was said to be present in the primary position while the left eye is
normal. She was then referred for evaluation of this fixing. Ductions were normal in the right eye.
traumatic left hypertropia, which was thought by her Depression of the left eye was limited more in abduc-
referring physician to be caused by a blowout fracture tion than adduction. On attempts to look down with
in spite of normal radiographic findings. the left eye, an asymmetric skin crease developed in
the left lower lid approximately 12 mm below the lid
margin.
433
Chapter 16
Diagnosis
Traumatic disinsertion of the left inferior rectus penknife, and tree branch, causing trauma to the supe-
muscle. rior oblique tendon, trochlea, superior rectus, and
medial rectus. When treated in a timely manner, reat-
Treatment/Surgery tachment of the traumatically detached rectus muscle
Reattachment of the left inferior rectus muscle. is effective in restoring normal function. In long-
standing cases or in cases with disturbance of the
Comment orbital fascia, it may be necessary to recess the con-
The inferior rectus is the muscle most com- tracted antagonist to free local restrictions in addition
monly affected by traumatic disinsertion.* This may to reattaching the detached muscle. If an extraocular
be due to the fact that this muscle is the least well pro- muscle has been lacerated posterior to the insertion,
tected of the extraocular muscles, especially during the cut ends should be simply reapproximated muscle
forced lid closure and upward movement of the eye to muscle with repair of the muscle capsule and inter-
with the Bell phenomenon. Any of the extraocular muscular fascia. However, the more posterior the lac-
muscles could be injured and subsequently weakened eration, the more likely the patient is to have cicatri-
by trauma, depending on the unique nature of a given cial restriction and persistent strabismus.
injury. We have seen several patients who sustained
foreign body penetration of the orbit with objects
such as a store display hook, doorstop, pencil,
This 77-year-old man was struck in the left eye by the blade of a mowing
machine. Miraculously, the large blade cleanly disinserted his left inferi-
or rectus without causing any other damage except a slight contusion of
the right lower lid. This patient was seen approximately 3 hours after the
accident. Two hours later, with the patient under local anesthesia, the
inferior rectus was reattached. The patient was immediately restored to
single binocular vision.
*Helveston, EM, Grossman, RD; Extraocular muscle lacerations, Am J Ophthalmol 81(6):754-760, 1976.
434
Strabismus case management
Clinical picture
A B
History
This 79-year-old woman has had vertical was a mechanical restriction to elevation associated
diplopia for the past 1 1/2 years. It began immediate- with the left inferior rectus confirmed at surgery.
ly after her second (left eye) cataract was removed Although the Fresnel press-on prism was successful
and an intraocular lens implanted. She has been in eliminating her diplopia, it caused blurring of
wearing Fresnel prisms, which eliminate the diplopia vision in the left eye. Glass prism would have been
but cause annoying blurring of vision. an alternative, but at 12 prism diopters glass prism
creates a wide edge on the spectacle lens. Prisms are
Examination heavy; the total weight is similar (but the edge
Visual acuity with correction is OD 20/25-2 reduced) if the prism strength is divided between the
and OS 20/40-2. A 12 diopter base-up Fresnel prism lenses. At surgery, 2 ml of 1% xylocaine was inject-
is on the left lens. Prism cover testing shows 18 ed for 360 degrees subconjunctivally near the limbus
prism diopters of right hypertropia (left hypotropia) to provide anesthesia in the left eye.(see p 69). The
at distance fixation in the primary position. This left inferior rectus was recessed using an adjustable
increases in upgaze. There is moderate limitation of suture. After the inferior rectus was detached and
elevation of the right eye and more pronounced limi- reattached to the globe with the suture, the patient
tation of elevation of the left eye. Passive ductions was asked to respond to visual targets overhead while
were restricted to elevation in the left eye. she was supine on the operating table. In addition to
this subjective testing, during which she observed
Diagnosis horizontal and vertical ceiling tiles, cover testing was
Left hypotropia with diplopia after cataract also done. The inferior rectus was secured with a
extraction from left inferior rectus restriction. bow knot when the patient reported single vision and
when no shift was noted on the cover test. An hour
Treatment/Surgery later in the recovery room, prism cover testing was
repeated. When the patient continued to show no
Adjustable left inferior rectus recession.
shift with the cover test, the knot was tied off into a
Comment surgeons knot. The presumed cause of the inferior
rectus restriction was myopathic change of the left
This woman gives a typical history of diplopia inferior rectus secondary to the retrobulbar injection
after cataract extraction. The cause of her problem of anesthetic agent at the time of the cataract surgery.
435
Chapter 16
Childhood esotropia 3
Childhood exotropia 1
Skew deviation 2
436
Strabismus case management
437
Chapter 16
Clinical picture
History Treatment
This 60-year-old man had retinal detachment The patient was given new glasses with the
repair done on the right eye one year ago. Six months proper myopic correction and a total of 10 diopters
ago, he underwent vitrectomy in that eye because of base-down prism OD and 3 diopters base-in prism
multiple large floaters in the vitreous. Since the sec- OS. He was also told that surgery could be done if he
ond surgery, he has had constant diplopia. He is was not satisfied with prism treatment.
unable to drive at night, especially in the rain. His
right eye myopia increased after surgery, but he wears Surgery
undercorrecting glasses to lessen the effect of his The patient later decided to have surgical cor-
diplopia. rection because he was more comfortable with his
Examination new glasses. An adjustable right superior rectus
recession was done.
Visual acuity with his present glasses is OD
20/300- and OS 20/25. He is wearing glasses as fol- Comment
lows: OD -4.75+0.75 X 150 degrees 4 diopters of Passive duction testing at surgery demonstrated
prism base-down, and OS -2.50+0.50 X 35 degrees 4 restriction to depression of the right eye. This con-
diopter of prism base-up. Prism and cover testing in firmed the need to explore the area of the right supe-
the distance shows 3 prism diopters of exotropia and rior rectus. Scarring of the intermuscular membrane
10 prism diopters of right hypertropia. At near, meas- and anterior Tenons capsule was found in conjunc-
urements increased to 5 prism diopters of exotropia tion with an encircling band and a sponge that were
and 12 prism diopters of right hypertropia with left immediately behind the superior rectus insertion. The
head tilt. There is slight limitation of depression OD. sponge and the band were removed and the superior
Refraction of the right eye is -7.00+0.75 X 115 rectus muscle was recessed using an adjustable
degrees. With this, visual acuity improves to 20/60. suture. Although this patients eyes were aligned
Diagnosis postoperatively, he complains of a strain while
wearing his fully correcting spectacles. It is possible
Right hypertropia producing diplopia after reti- that the patient is actually having symptoms from the
nal detachment repair and vitrectomy of the right eye. aniseikonia, in which case treatment may be aimed at
intentionally blurring the right eye by undercorrecting
the myopia as was done before surgery. In some cases
with unequal visual inputs acquired in adulthood,
comfortable binocular vision is not attainable. This
may be such a case.
438
Strabismus case management
C A D
A, Chin depression with straight eyes; B, esotropia in downgaze; C, overaction of the right inferior oblique;
D, overaction of the left inferior oblique; E, eyes aligned in upgaze.
History
This 4-year-old girl was brought in for exami- marked (3+) overaction of both inferior obliques. A
nation by her parents because they observed that she V pattern was present with straight eyes when look-
holds her chin down when she looks at things and that ing up and 35 prism diopters of esotropia when look-
one eye seems to shoot up when she looks to the ing down. The remainder of the examination was
side. unremarkable.
Examination Diagnosis
Marked chin depression was the most obvious 'V' pattern esotropia with bilateral overaction of
sign when observing this child. She had 20/40 vision the inferior oblique muscles.
in each eye with pictures. Cyclopegic refraction was
OD +1.75 and OS +1.50. Versions demonstrated
439
Chapter 16
Treatment/Surgery
Bilateral inferior oblique weakening. pattern by decreasing the esotropia in downgaze. As
an alternative, the medial recti could be shifted
Comment downward to treat the V, but this would be more
This is a case of so-called primary overaction of reasonable if there were anti-mongoloid fissures or
the inferior obliques. Such a case could be called other evidence of pulley heterotopy. In case of a
bilateral congenital superior oblique palsy. However, bilateral congenital superior oblique palsy confirmed
in this case, at surgery the superior oblique traction by finding a loose superior oblique tendon on superi-
test was normal bilaterally, as indicated by finding a or oblique traction testing, the superior oblique
definite knife-edge response. The true cause for weakening procedure could be done. This procedure
this so-called primary inferior oblique overaction is is more difficult. Most surgeons prefer to weaken the
unknown to me. In a case like this, bilateral inferior inferior obliques.
oblique weakening is effective in opening up the V
B A C
This patient is similar to the previous patient with a V pattern except that A, the head posture is normal; B, and
C, the inferior obliques do not overact; and D, a small exotropia is present in upgaze and E, Esotropia is present
in downgaze. This patient would benefit from downward shift of the insertion of the medial rectus muscles.
440
Strabismus case management
Clinical picture
C A D
A, In the primary position the eyes are aligned with the chin slightly depressed. B, A small esotropia is seen in
upgaze. C, The left superior oblique overacts. D, The right superior oblique overacts. E, Fifty diopters of
exotropia is seen in downgrade.
History Examination
This 19-year-old college freshman has had dif- Visual acuity without correction is 20/20. The
ficulty in concentrating on her schoolwork. She has eyes are straight in the primary position, but the
difficulty especially when required to read for long patient appeared to dip her chin down slightly and to
periods. She is also concerned because her friends look upward as an unconscious gesture. Six of nine
often say that there is something funny about her stereo dots (80 seconds) were fused. Ductions were
eyes. Neither the patient nor her parents had any full in either eye. On testing of versions, a significant
apparent further insight into the problem in that they A pattern was observed with bilateral overaction of
were not aware of any crossing or misalignment of the superior oblique muscles. Cover and uncover
the eyes. testing revealed 1+ dissociated vertical deviation in
each eye in the primary position. Five prism diopters
441
Chapter 16
of esotropia was present in upgaze and 50 prism aware that something was wrong. In addition, this
diopters of exotropia was present in downgaze, con- patient is doing satisfactory schoolwork with only
firming that a large A pattern was present. These minimal symptoms. However, if in the future, she has
motility findings were demonstrated to the girls par- more difficulty with either her schoolwork or with the
ents. They exclaimed that they had never seen the comments of her friends, surgery could be done. The
eyes do this! fact that both the patient and her family will have
some insight into the nature of the strabismus makes
Diagnosis it more reasonable to consider surgery some time in
The triad-A exotropia, bilateral overaction of the future. It is not a good idea to perform surgery for
the superior obliques, dissociated vertical deviation.* a strabismus that neither the patient nor the family
noticed!
Treatment If surgery were to be done for this patient, it
None now -- see comment. would consist of bilateral weakening of the superior
oblique tendons. It should be understood that weak-
Surgery ening of the superior oblique tendons in a fusing
patient should be done symmetrically and with full
None now -- see comment. consideration for the consequences; that is, loss of
Comment fusion and creation of vertical strabismus with
diplopia. In other cases of this triad where the disso-
This patient demonstrates an ocular motility ciated vertical deviation is also a significant problem,
triad that is not uncommon but which must be looked bilateral superior rectus recession can be combined
for carefully and with awareness that such a pattern of with bilateral superior oblique weakening. As an
motility disturbance exists. This triad can occur pri- alternative procedure in this case, the lateral rectus
marily or as a secondary strabismus. It can occur in a muscles could be shifted downward one-half to three-
patient who has excellent fusion or in one without quarters muscle width. This procedure is safer and
fusion. The degree of involvement can be mild or it is certainly easier to reoperate if the need should
marked. In this case, the patient had only minimal arise. It would treat the superior oblique overaction if
DVD but had a rather marked A pattern. She was the lateral pulleys were displaced upward as in a
able to compensate for this effectively by assuming a mongoloid fissure.
slight chin depression. In cases like this, it is often The 5-year-old boy shown below was examined
necessary to point out the findings to the family. For because he habitually kept his chin down and looked
this patient, I recommend no surgery for several rea- up. Because this was becoming a problem, according
sons. First, neither the patient nor her family knew to his parents, and since they were aware of the stra-
that there was any specific motility problem when bismus and the implications of treatment, he was
they came for this examination. They only were treated with bilateral superior oblique recession.
A B C
A, With his chin down and looking up, this patients eyes were aligned. B, Exotropia measuring 40 diopters in
downgaze. C, Aligned eyes in upgaze.
* Helveston, EM: A-exotropia, alternating sursumduction and superior oblique overaction, Am J Ophthalmol 67:3, March 1969.
442
Strabismus case management
Clinical picture
History Diagnosis
This 28-year-old woman was treated for a Parinaud syndrome--paralysis of upgaze.
pituitary tumor at age 5 years. Since that time, she
has been unable to look up. In order to see, she Treatment/Surgery
chronically elevates her chin and looks down. She Recession of both inferior recti 5.0 mm, resec-
is also bothered by intermittent diplopia. Her tion both of superior recti 6.0 mm, with advance-
biggest problem now is pain and stiffness in her ment of Lockwoods ligament 5.0 mm.
neck, presumable secondary to chronically elevating
her chin. She frequently seeks professional help for Comment
the neck pain. This patient has no realistic hope of fusion, but
Examination she can be helped by simply bringing her eyes up to
the primary position to enable comfortable vision
Visual acuity is OD 20/25 and OS 20/40 while without elevating her chin and arching her neck. In
wearing the following correction: OD +1.25+0.75 X this type of case, expectations are limited, but any
135 degrees, and OS +1.75+0.50 X 45 degrees. The help that the patient can obtain is usually greatly
patient assumes approximately 20 degrees of chin appreciated. After surgery, this patient had no need
elevation while looking downward chronically. She to lift her chin, but she was slightly more aware of
cannot elevate her eyes even to the midline. The diplopia. She considered this a more than adequate
eyes are approximately 15 prism diopters exodeviat- trade off for the improved head and neck posture.
ed in downgaze and approximately 15 prism
diopters esotropic with maximum attempt of upgaze.
443
Chapter 16
Clinical picture
A B
A, With the head straight acuity is diminished and nystagmus is present (vision 20/80); B, With right face
turn and levoversion nystagmus is dampened (acuity 20/40).
History Treatment/Surgery
This 29-year-old woman complains that her Anderson procedure: Recession of the left lat-
eyes dance and her vision is poor. The only way she eral rectus, and recession of the right medial rectus.
can improve her visual acuity is to turn her face to the As an alternative this patient could have recession of
right and look far to the left. She is employed as a the four horizontal recti to the equator.
technician in an ophthalmologists office.
Comment
Examination
Since the description more than a half century
With the head straight and the eyes in primary ago, the surgical procedure of choice for null point
position, visual acuity is 20/80 in each eye and with nystagmus has been based on the principle of the
both eyes open. A large amplitude right-beating nys- Kestenbaum-Anderson procedure. This procedure
tagmus is present. When the patient attempts to attempts to shift the null point of nystagmus to the
improve her visual acuity, she turns her face 40 straight-ahead position. To accomplish this, using as
degrees to the right and assumes maximum levover- an example the patient shown here, the left lateral and
sion. Visual acuity then is 20/40 with both eyes open. right medial rectus muscles are recessed and left
Visual acuity in levoversion reduces to 20/60 with medial and right lateral rectus muscles are resected.
either eye occluded, because of latent nystagmus. This means that the neural output for levoversion
After repeated checks of visual acuity, the patient required preoperatively will put the eyes in the pri-
always assumes the same head posture with right face mary position with the head straight or nearly straight
turn and left gaze. The remainder of the eye exami- after surgery. In the years since its inception, this
nation is unremarkable. type of surgery has been only moderately successful.
Actually, a few years after surgery, most patients note
Diagnosis that the head posture gradually returns or they find
Null point nystagmus. that they can achieve comfortable vision by turning
their head just as far as before but in the opposite
444
Strabismus case management
direction! In the beginning, Kestenbaum recom- amplitude of nystagmus diminishes by about 50%. If
mended recessions and resections limited to 5 mm. the null point nystagmus improves and then later
Anderson recessed only the two yoke muscles a dis- reverts to preoperative findings or if no improvement
tance of 5 mm. Since this null point nystagmus sur- results from large recession of the four horizontal
gery tended to be unsuccessful, the surgical amounts recti, the yoked rectus muscles opposite the preferred
were gradually increased proportionally by frustrated version (the same side as the direction of the face
surgeons. Finally, Pratt-Johnson described doing 10 turn) are advanced to their original insertion. This
mm recessions and resections in both eyes. I suspect procedure(s) appears to be more physiologic and def-
that even this maximum surgery will over time fail in initely is tissue sparing. Actually it is a larger, staged
many cases. There is something decidedly nonphysi- Anderson procedure. At the present time, this
ologic about this null point nystagmus surgery, approach to null point nystagmus is unproven.
because after this surgical procedure is done, patients However, since the Kestenbaum-Anderson procedure
must chronically exert effort to hold their eyes and is proven, but proven lacking for the most part, any
head straight, whereas in the normal state this posture logical alternative for this surgery seems reasonable.
should require the least effort! This may be the rea- In some cases of null point nystagmus, a pri-
son why some patients assume a head posture in just mary Anderson procedure can be done using larger
the opposite direction in order to sustain comfortable numbers than originally described. For example,
vision. with null point achieved by dextroversion the right
I have begun to do recession of the four hori- lateral rectus would be recessed 8.0 to 10.0 mm and
zontal recti instead of the modified Kestenbaum- the left medial rectus recessed 12.5 mm from the lim-
Anderson procedure for most null point nystagmus. bus.
By relaxing all of the horizontal rectus muscles, the
445
Chapter 16
Clinical picture
History Diagnosis
This 16-year-old boy has been followed for Congenital motor nystagmus with decreased
congenital nystagmus since age 5 years. He and his visual acuity.
mother say that he has had dancing eyes all of his
life. Visual acuity had been recorded on repeated Treatment/Surgery
occasions with the letter chart at no better than 20/80 Recession of the four horizontal rectus muscles.
at distance with both eyes. He has always been able
to read newspaper size print and he has made satis- Comment
factory progress in school. However, because of Nystagmus has two important connections with
reduced visual acuity, he was unable to obtain a learn- visual acuity. First, poor vision causes nystagmus, if
ers permit and enroll in drivers education. The boy the poor vision has onset at a young age. The 2-4-6
and his mother returned for an annual examination at rule of Cogan states that poor vision occurring before
age 14 years to find out if anything could be done to 2 years of age always produces nystagmus, poor
help this boys acuity level that would allow him to vision before 4 years of age can result in nystagmus,
obtain his learners permit. He drives a motorbike and poor vision after 6 years of age does not produce
without difficulty and has driven an automobile under nystagmus. The second important relationship is that
supervision with his family. nystagmus causes reduction in vision. These two can
Examination be combined. That is, nystagmus can cause a reduc-
tion in visual acuity in an otherwise normal eye or in
Before surgery, visual acuity with correction is an eye with some reduction in vision because of the
OD 20/80 and OS 20/100, both eyes 20/80 without motion of the retinal image. In a case where suitable
glasses. Cycloplegic retinoscopy is OD and OS retinal potential exists, stabilizing the retinal image
+0.75. A rapid, moderate amplitude pendular nystag- results in improved visual acuity. A common strategy
mus is present in the primary position. This becomes for stabilizing the retinal image and improving visual
a right-beating nystagmus on right gaze and a left- acuity is the head turn adopted in null point nystag-
beating nystagmus on left gaze. The nystagmus mus. In another strategy, nystagmus can be reduced
amplitude increases slightly in each eye when the fel- and visual acuity improved by convergence. Most
low eye is occluded. Near vision is 20/40, and the patients with congenital motor nystagmus and with
nystagmus is damped significantly by convergence. nystagmus having its origins in relative visual deficits
Both optic nerves are small, but no double rings sign will see better at near because of reduced amplitude
is noted and a clear cut diagnosis of bilateral optic of nystagmus and because the retinal image is larger.
nerve hypoplasia cannot be made. The remainder of Nystagmus can also be damped by induced
the eye examination is unremarkable. convergence stimulated by the use of base-out prism,
*Speilmann A, Dahan A: Double torticollis and surgical artificial divergence in nystagmus, ACTA Strabol, 1985 p 187.
446
Strabismus case management
by overcorrecting minus lenses and by surgery to pro- tion of nystagmus amplitude with or without
duce artificial divergence as recommended by improvement in visual acuity.
Spielmann*. However, this treatment technique has 3. If visual acuity improves at all after surgery,
not become widespread. Surgery to shift the null it is on the order of one line!
point of nystagmus (Kestenbaum-Anderson proce- 4. Recession of the four horizontal recti is car-
dure) does not improve vision; it simply attempts to ried out to or slightly in front of the equator.
improve head posture in a patient who has already 5. After surgery, nystagmus amplitude is
developed a strategy to improve vision. reduced about 50% while frequency remains
The four-muscle recession procedure is a unchanged.
unique strategy for damping nystagmus and in turn 6. As pointed out by Sprunger, et. al., recogni-
improving visual acuity. This was originally done 40 tion time improves after four muscle reces-
years ago by Bietti and Bagolini. They discarded the sion. This means that a patient can recognize
technique because it did not retain its effectiveness. the smallest optotype in less time. The func-
Later, Limon of Mexico City revived the procedure tional value is that a person can enter a new
and reported significant success. von Noorden and and possibly confusing environment and
Sprunger reported successful results in three patients, become oriented faster.
and we later reported successful results. 7. After surgery, ductions are reduced, but sym-
The principle of the four-muscle recession is to metrically and to a minimal degree.
more or less diminish the power of the rectus muscles 8. New strabismus after four muscle recession
by reducing the length tension and the lever arm in a is rare and is easily managed at a second pro-
balanced way so as to not induce strabismus. This cedure.
technique is designed to reduce the exuberance of the 9. Patients are pleased with the results in nearly
contraction of the muscle, the factor ultimately every case and state that they would do it
responsible for the eye movement. again.
The technique for recession of the four hori- 10. On three occasions, a second family member
zontal recti for treatment of nystagmus was original- underwent the surgery after observing results
ly to move the muscles back to a point approximate- in a parent, a sibling,, and a grandparent. A
ly 2 mm behind the equator. Some surgeons moved woman who had surgery for her nystagmus
the medial and lateral rectus muscles a nearly equal brought her son a year later; she was so
amount meaning that the relative recession of the pleased with the results. In another case it
medial rectus was actually greater. As would be was sisters with universal albinism. The sec-
expected, this produced exotropia pointing out the ond sister, observing the results of surgery,
need to recess the recti a proportional amount mean- accompanied her sister at a one year follow
ing that the medial recti should be recessed 2 or 3 mm up and requested surgery for herself. In a
less than the lateral recti. Later it was suggested that third case, a grandfather with nystagmus had
since the functional origin of the rectus muscles could the surgery to see if it was effective. After
be at their pulley, recession behind the equator was the results of surgery were evident, he
not necessary. In response, I began recessing the hor- brought into the clinic two grandchildren to
izontal only to the equator with no change in results have four muscle recession.
from surgery. Recently, Hertle and DellOsso reported that
Indications for four-muscle surgery for nystag- simply detaching and reattaching the four horizontal
mus are the following: rectus muscles has a beneficial effect on nystagmus.
1. Reduced visual acuity and nystagmus, This would rely on the disruption of proprioceptive
preferably, but not necessarily, with vision response of the rectus muscles.
improved with the nystagmus damped. The four muscle recession for treatment of nys-
2. Difficult eye contact because of nystagmus tagmus is said to rely on soft evidence and anecdot-
3. Understanding on the part of the patient that al endorsement, making it difficult to defend on a
improvement will be incremental and not purely scientific basis. In addition, it was subject to
necessarily dramatic criticism and even warnings against what could be a
4. No contradiction to surgery wholesale approach to the treatment of nystagmus.
My experience with four muscle recession for This has not happened. Instead, a relatively small
the treatment of nystagmus can be summarized in the number of patients have received a modest but appre-
following: ciated improvement in both their visual function and
1. Less than 10% of nystagmus patients fit the appearance.
criteria for surgery. Patients should be thoroughly counseled before
2. Patients who are candidates for surgery this surgical procedure is done. The small average
should be expected to benefit from the reduc- improvement of visual acuity should be emphasized.
447
Chapter 16
Patients should be told that the nystagmus will not go mus, it is clear that the four-muscle recession is less
away. At best, the amplitude is reduced by approxi- radical than other methods for surgical treatment of
mately 50% but the frequency remains unchanged. nystagmus, some of which have been more or less
Patients are also told that between 10% and 20% of accepted as standard and routine.
patients need additional surgery for a new horizontal Immediately after the recession of the four hor-
strabismus, usually an exotropia. In our practice, izontal recti, visual acuity in the patient described in
6.5% of 396 consecutive patients seen over a 5-year this case improved to 20/60 with both eyes open. On
period with a principal diagnosis of nystagmus had the basis of this improvement, he was able to obtain
this type of surgery. This means that because of strin- a drivers license with driving privileges limited to
gent selection criteria, relatively few patients are con- the daylight hours. Two years later, vision remained
sidered suitable candidates for four-muscle recession at 20/60 tested binocularly, but 20 diopters of left
for the treatment of nystagmus. When comparing exotropia developed. This was treated with a 6.0 mm
four-muscle recession, a tissue-sparing operation, advancement of the left medial rectus, which result-
with the bilateral 10 mm recession/resection or sim- ed in alignment.
ilar procedures, for treatment of null point nystag-
A B C
A, Primary position alignment after four horizontal rectus recession posterior to the equator followed by 6.0 mm
advancement of the left medial rectus for secondary exotropia. B, Dextroversion is full. C, Levoversion is full.
448
Strabismus case management
Treatment/Surgery
Retrobulbar Botox.
Comment
This is the second patient ever to be treated
with retrobulbar Botox. I treated the first patient
with toxin injected into the four rectus muscles. This
had no beneficial effect. As a second attempt in this
patient, 25 units of Botox was injected into the retrob-
ulbar space. This patient had improvement of visual
The right eye has vertical, horizontal, and rotary nystag- acuity from less than 20/100 to 20/30 in 2 days. The
mus with oscillopsia after a brain stem stroke. The left improved vision resulting from decreased movement
eye vision is hand motion from a corneal scar. A left
of the eye lasted for about 3 months. After that, the
seventh nerve palsy persists. A lateral tarsorraphy has
been done. Botox injection had to be repeated, fortunately pro-
ducing similar results. After three injections, this
patient asked to be followed by an ophthalmologist
History closer to her home to continue this form of treatment.
The patient described here, the second patient
This 55-year-old woman complained of so treated, had the same response as the patient
reduced vision because of constant movement of her described in the preceding paragraph. She has had a
environment (oscillopsia). She had a brain stem total of more than 30 retrobulbar Botox injections,
stroke 2 years previously, and in addition to her visu- each remaining effective for approximately five
al difficulties, she is confined to a wheelchair because months. After each injection, visual acuity improves
of hemiparesis. Her left eye has only hand motion to between 20/30 and 20/40 within 24 to 48 hours.
vision because of a corneal scar following an ulcer No complication from this treatment has been noted.
that occurred secondary to exposure, which in turn At first we were curious that this patient never had
was caused by lagophthalmos from seventh nerve ptosis after any treatments, nor did the first patient
palsy on the left. This patient wanted some relief after three treatments. We later learned that this was
from oscillopsia to be able to read and to watch tele- because we had the patients sit up immediately after
vision. the injection. This prevented the toxin from pooling
at the apex and affecting the nerve to the levator
Examination palpebri. Because of presbyopia in both of these
Visual acuity in the right eye with correction of patients, requiring reading glasses, any effect on
-0.50+1.50 X 90 degrees is 20/100 and in the left eye accommodation is not significant.
it is hand motion. A large amplitude pendular hori- More than two dozen patients have been treated
zontal vertical and to a lesser extent rotary nystagmus successfully in our clinic. The relief is temporary, but
is present in both eyes. The left eye is 30 prism is appreciated by the patients.
diopters esotropic, with a dense corneal scar obscur- Retrobulbar Botox provides significant tempo-
ing the visual axis. A facial paralysis is present on the rary improvement in vision for the few who qualify
left as well as sixth nerve palsy on this side. The right for this type of treatment.
eye is normal except for the nystagmus.
449
Chapter 16
Clinical picture
History Surgery
This 47-year-old teacher complains that sever- Right superior oblique weakening plus ipsilat-
al times a day he sees things double and moving. eral inferior oblique weakening
The second image moves with a pulsating torsional
pattern. Without warning, objects seen by the left Comment
eye tilt upward and outward. He can stop the sensa- Superior oblique myokymia is a rare ocular
tion only by closing this eye. It disappears sponta- motility disorder. In the handful of cases that I have
neously and comes on without warning. seen, patients are extremely disturbed by the oscil-
lopsia. Medical treatment may not be successful and
Examination can have serious side effects. I do not endorse it.
The patient has 20/20 visual acuity in each eye. Superior oblique weakening can be successful in alle-
The remainder of the eye examination is completely viating these symptoms. I prefer to do this by means
normal, and he fuses 9/9 stereo dots (40 seconds). of disinsertion of the tendon. Superior oblique palsy
Refraction is plano in each eye. After observing this occurs frequently in cases treated this way. When
patient for several minutes and after repeated testing this occurs, a weakening procedure of the antagonist
of versions, especially having him look up and to the inferior oblique is done. I have treated one case of
right, a rhythmic incyclodeviation and depression of superior oblique myokymia with injection of Botox
the right eye began. This is a motion typical of supe- into the superior oblique muscle. This also produced
rior oblique myokymia and nothing else. While I a marked ptosis. When the ptosis resolved, the
observed these motions, the patient described the myokymia returned. A repeat injection did the same,
excyclo-oscillopsia. but the myokymia was greatly improved after the
second injection. Whether this is only due to the
Treatment Botox treatment is open to question.
Just wait. It may go away! Possible medical
treatment includes carbamazepine initially. Also,
clonazepam, phenytoin, and baclofen have been
used.
450
Strabismus case management
Clinical picture
A B
A, Esotropia of 45 prism diopters. B, Eyes are aligned wearing +3.00 diopters spectacles.
Comment
This boy, who is now 5 years old, developed A disquieting movement advocating surgery
esotropia at age 3 years. He was found to have a instead of optical correction for refractive esotropia
hyperopia of +3.00 diopters and given glasses to fully has begun among some strabismologists, primarily in
correct this. While wearing these glasses, he is Europe.* Substituting surgery for optical correction
aligned with 9/9 stereoacuity (40 seconds) and has for refractive esotropia flies in the face of all that we
essentially normal motility. This represents a typical know about the nature of this condition. Patients
pattern for a patient with refractive esotropia. After undergoing surgery for refractive esotropia continue
initial success, these patients typically continue to do to require correction for hypertropia and are therefore
well with glasses wear. Some patients are even able asthenopic or simply have blurred vision without
to revert to part-time wearing of glasses during the optical aid, particularly in the teens and later years.
teen years and later, but because of asthenopia and/or In addition, patients treated with bimedial rectus
blurred vision related to the hyperopia, they always recession or equivalent for refractive esotropia are
return to wearing glasses during adulthood and well very likely to develop exotropia with or without rein-
before the usual age of presbyopia. These patients stitution of glasses. Surgery in place of hyperopic
have a normal to slightly high accommodative con- correction for treatment of refractive esotropia
vergence--accommodation ration (AC/A), and the should be condemned!
deviation is the same at distance and near.
*A roundtable discussion on the management of fully accommodative esotropia can be found in Campos E, Editor: Strabismus and ocu-
lomotility disorders, Proceedings of th e Sixth Meeting of the International Strabismological Association, London, 1991, Macmillan, pp
269-305.
**Jampolsky A, von Noorden GK, Spiritus M: Unnecessary surgery in fully refractive accommodative eostropia Australian-New
Zealand. J Ophthalmol Nov 1991 (19)4 p 370-373.
451
Chapter 16
Clinical picture
B C
A, Without correction, this patient has esotropia at distance (in some cases of pure accom-
modative esotropia [high AC/A] the eyes may be aligned in the distance) and crossing at
near. Her refraction after cycloplegia is OD and OS +2.50. B, Fixing in the distance
through the carrier and wearing full correction, the eyes are aligned. C, Fixing at near
through the carrier, the patient has a large esotropia. D, Fixing at near through the +3.00
add (bifocals are usually from +1.50 to +3.50), the eyes are aligned.
Comment
Refractive/accommodative esotropia can be bifocals at all. In selected cases, bimedial rectus
difficult to manage. These patients may be plano, recession with or without posterior fixation suture
hyperopic, or even myopic, but they all have in com- may be done for patients whose eyes are straight in
mon a high accommodative convergence/ accommo- the distance but who reach their teen years and cannot
dation (AC/A) producing an esotropia at near. The be weaned from bifocals without developing
philosophy of treatment varies according to the stra- esotropia at near. There is no universally agreed upon
bismologists experience. I use bifocals if the eyes way to deal with this type of strabismus. Prolonged
are aligned at near while looking through them, but wearing of bifocals has been said to contribute to pre-
not if the angle of deviation is only reduced. Patients mature presbyopia. This could be a reason for dis-
may be continued with bifocals as long as they are continuing them in favor of surgery or even allowing
necessary to maintain fusion. Some strabismologists the child to be esotropic at near. In such a case, the
attempt to wean patients from bifocal wear gradually, distance alignment can have an effect on the near con-
while others stop bifocals abruptly at a certain age or tributing eventually to near alignment.
at a certain reduced near deviation. Some do not use
452
Strabismus case management
Clinical picture
A B
C D
A, Esotropia in a 6-month-old girl. B, Eyes aligned with +3.00 diopter glasses. C, Without correction, the
esotropia remains at age 6 years. D, With glasses, the eyes are aligned but DVD is present in each eye.
Comment
This infant was brought in for examination at straightened with glasses instead of surgery. The
6 months of age with 30 diopters of esotropia that best treatment for this child is the continued use of
had its onset at 3 months of age. Full correction of her glasses. She was later found to have asymmetric
3.00 diopters of hyperopia in each eye resulted in optokinetic nystagmus suggesting that the basic
straight eyes. This is early for refractive esotropia. problem is a congenital fusion deficit. To further
By age 4 years, this patient, who retained alignment complicate this case, the girl was eventually found
while wearing her glasses demonstrated gross stere- to have overaction of the superior obliques causing
opsis, but also had DVD! This case blurs the dis- an A pattern. This case may be an example of very
tinction between refractive and congenital esotropia. early onset refractive esotropia actually being a
I believe that this child has congenital esotropia milder form of congenital esotropia
453
Chapter 16
Summary
The 67 case histories included here are a sam- inferior rectus muscles. Since that time, imaging of
ple of the clinical spectrum of strabismus. Because of the orbit has shown that this deviation could be
its unlimited potential for variation, strabismus could caused by a migration of the lateral rectus inferiorly
never be comprehensively described. This list instead secondary to a dehiscence of the superior temporal
offers a glimpse of the larger clinical picture. There intermuscular membrane due to the enlarging globe.
will undoubtedly be omissions noted. For example, I This makes the inferior rectus a depressor. In this
have not discussed inferior oblique palsy, a condition case, elevating the lateral rectus and even connecting
seen one or two times a year in a busy strabismus it to the superior rectus muscle has been suggested
practice. In order to avoid overlooking this entirely I along with medial rectus recession. I have had no
would simply suggest recession of the yoke, the con- experience with this treatment
tralateral superior rectus, in this case. A tuck (or A strabismus surgeon will encounter clinical
resection and advancement) of the paretic inferior problems that differ from those presented here in
oblique would make sense, but this procedure is detail but not necessarily in kind. Because each case
rarely done. is unique, you will be required to provide your own
Another case not described in this list of cases personal solution for the management of each strabis-
is the heavy eye hypotropia and esotropia that mus patient. In doing this, you should always strive
occurs in some patients with very high myopia on the to manage each problem by using tested principles
order of approximately -15.00 and above. Such a and by adhering to sound surgical technique. You
patient was seen early in the telemedicine program should apply the tools, skills, and insights of a pro-
when patients were submitted via e-mail. Pictures of fessional challenged with the need to solve a wide
this patient were given to Dr. von Noorden for the array of complex problems.
sixth edition of Binocular Vision and Ocular Motility If this book has any value, I hope the value is
and can be found on page 474 with an excellent that it challenges the strabismus surgeon to arrive at
description of this condition. My advice to the refer- the proper approach to a strabismus problem through
ring doctors in 1999 was to recess the medial and the application of sound principles rather than by
adherence to dogma.
454
17
Complications of
strabismus surgery
Complications of strabismus
surgery
In the performance of surgery for strabismus or
place. An example of this type of complication is
any other indication, complications will occur if sur-
lower lid lag with widened palpebral fissure after
gery is performed in a sufficient number of cases.
inferior rectus recession. (3) The third category of
This means that surgical complications can be elimi-
complication is characterized by a new problem that
nated, at least on a statistical basis, only by not doing
is unrelated to the reason for the planned strabismus
surgery. Therefore, it is a truism that if a surgeon per-
surgery. This type of complication would occur, for
forms surgery enough times, a complication will
example, if the patient is burned by an anesthesia-
occur. However, whether or not a complication is
heating device or if a retinal detachment or endoph-
said to occur depends in part on the surgeon's defini-
thalmitis occur.
tion. The only certain way to avoid complications
The results of strabismus surgery are unique
other than to avoid surgery is to deny that a compli-
because they are clearly evident for all to see. They
cation exists. This self-serving strategy on the part of
are neither covered by clothing nor internally located
the surgeon is seldom tolerated by the patient.
(although dark glasses may be used). After strabis-
For our purposes, a complication is defined as a
mus surgery patients can, and do, study their align-
factor or event developing in the course of treating a
ment by looking in the mirror and by testing them-
primary condition that appears unexpectedly and
selves for diplopia. They also react to how others
changes existing plans and/or outcome. Just what
seem to relate to them ("people don't know where I'm
specifically constitutes a complication varies from
looking"). The patient also evaluates the results of
surgeon to surgeon. The following are criteria estab-
surgery on a functional basis reporting, for example,
lished from my personal experience.
diplopia or asthenopia.
Complications range from minor and annoying
to severe and threatening. The latter challenge the Criteria for success after
well-being of the patient and create extra concern on
the part of the surgeon. Complications occur in three
strabismus surgery
categories. (1) Unacceptable results are complica- To establish a better foundation for evaluation of
tions that relate directly to the reason for the surgery. complications, it is appropriate to discuss criteria for
The results are unsatisfactory because the alignment success. A perfect result from strabismus surgery
is not cosmetically acceptable, diplopia, persists, the could include the following: (1) minimum immediate
conjunctiva is scarred and unsightly, or similar rea- postoperative discomfort, (2) no apparent conjuncti-
sons. The surgeon is not happy with the effects of val scars, (3) normal palpebral fissures, (4) normal
surgery because he thinks he could have produced a versions and ductions, (5) orthotropia, (6) equal and
better result. (2) A second category of complications normal visual acuity, and (7) normal stereo acuity.
is a new problem related to the reason for surgery. Only rarely is the perfect result attainable because
Although the problem may be related to the general the patient who requires strabismus surgery rarely has
area of strabismus surgery, it is not necessarily relat- the motor and sensory potential for attaining such a
ed to the condition being treated surgically in the first result, except perhaps for the patient who has inter-
455
Chapter 17
mittent exotropia and normal stereo acuity. In prac- acted to the right amount of surgery." Although this
tice, although the intermittent exotropia patient is said primarily in jest, there is a grain of truth pres-
potentially may be the one most likely to attain per- ent. A case in point is iatrogenic Brown syndrome
fect results, often these results are not achieved. This that can occur after a tucking or other strengthening
may be for the same reason that the patient develops procedure carried out on the superior oblique tendon.
intermittent exotropia in the first place. The patient may achieve an excellent effect in the pri-
If a perfect result is not attainable after strabis- mary position but develop diplopia in the field of
mus surgery, what then? A reasonable goal for stra- action of the antagonist inferior oblique muscle
bismus surgery should be established to determine a because of a non-yielding superior oblique tendon.
basis for what could be termed an acceptable result. The question is, "Was the surgery performed proper-
For example, an acceptable long-term result could ly?" From the patient's point of view, the answer
provide the patient with improved function and/or might be no. From the surgeon's point of view, how-
appearance without the introduction of other compli- ever, the surgery may have been performed exactly as
cating factors. Some acceptable results could include planned, but too much of the tendon was tucked.
small undercorrection, small overcorrection, minimal Although preoperative superior oblique tendon test-
dissociated vertical deviation, a slight or barely per- ing leading to titrated superior oblique tuck makes
ceptible lid fissure anomaly, slight incomitance, min- this condition less frequent, it still occurs. In either
imal conjunctival redness, diplopia only in extreme case, the patient has a problem and relief is needed.
gaze, and mild Brown syndrome, to name a few. Late-occurring exotropia or dissociated vertical devi-
Early postoperative phenomena that are self-limited ation (DVD) after initially successful surgery for
and should not be considered complications include: esotropia should not be considered a complication,
transient suture reaction, benign subconjunctival but rather an unstable result in a patient who does not
hemorrhage, minimal lid edema, corneal abrasion have bifoveal fusion potential. It is a manifestation of
with prompt healing, and a delle with prompt healing. the natural history of the strabismus.
An acceptable result can include a variety of less than Some new problems related to strabismus sur-
perfect results and even some transient complica- gery could include the following: lost muscle, infe-
tions that end in the best result that can be hoped for rior oblique adherence or inclusion syndrome, scleral
given the presenting condition. perforation, retinal detachment, orbital hemorrhage,
The above lists are relatively minor inconven- cellulitis, operation on the wrong muscle, proptosis,
iences or discomforts that may or may not be avoid- symblepharon, conjunctival cyst, Tenon's prolapse,
able. Since they are completely reversible, they may endophthalmitis, surgical procedure on the wrong
not be considered a complication. The status of eye, or surgical procedure on the wrong patient.
stereo acuity, fusional amplitudes, and vision are pre- New problems appearing after strabismus sur-
operative modifying factors. They can affect the out- gery that are unrelated to the original surgery can be
come of surgery but are neither ordinarily affected, the most serious of all complications and include pro-
nor enhanced by, proper surgery. longed apnea, hyperthermia, gastric bleeding, and
In assessing the results of strabismus surgery, a even death. It should be noted that the unrelated or
very important axiom should be remembered: noth- new problems associated with strabismus surgery are
ing gets better with litigation pending. The person commonly anesthesia-related. Prolonged apnea may
who is involved in an accident and who is seeking occur in patients who have received a depolarizing
compensation or other type of relief may depend relaxant such as succinylcholine in the presence of
either consciously or unconsciously on pain and suf- reduced blood pseudocholinesterase levels, such as
fering to achieve a perhaps deserved amount of com- would occur after treatment with phospholine iodine.
pensation. It can be more difficult for such a patient Unplanned admission of an outpatient, usually relat-
to maintain a positive attitude toward obtaining a ed to an anesthetic complication, or vomiting, may be
good result from a surgery. The surgeon must be reported as a complication by the hospital's quality
patient, supportive, appropriate, and above all, under- assurance committee.
standing in such cases. Malignant hyperthermia is a familial condition
Complications after strabismus surgery can but is difficult to anticipate unless a positive family
include constant diplopia, unsightly conjunctival history is noted. Most operating rooms are now sup-
scars often with limitation of ductions, overcorrection plied with dantrolene, which is administered immedi-
of a significant amount according to the criteria of the ately when malignant hyperthermia is encountered.
patient and surgeon, undercorrection of a like I encountered an unusual complication of sur-
amount, significant lid fissure anomalies, and severe gery, severe postoperative gastric bleeding. This
Brown syndrome. Some of these problems can occur occurred in a patient who apparently had an aberrant
in spite of properly performed surgery. In the case of artery at the esophageal-gastric junction. Bleeding
an overcorrection, the strabismus surgeon could say: developed after this artery ruptured with postopera-
"Mrs. Jones, your surgery went fine, but you overre- tive suctioning of the stomach. An overnight admis-
456
Complications of strabismus surgery
sion was required, but the bleeding stopped without A few patients may have a type of diplopia that
specific treatment. A skin burn from a heating blan- is not a complication of surgery but is the patient's
ket and one case in which a heated tube on the anes- own problem. This type of diplopia has been termed
thesia machine caused a second-degree thermal burn central disruption of fusion that can occur after closed
of the arm are other examples of complications unre- head trauma. Other patients have foveas that repel
lated to strabismus surgery. rather than attract with a condition called horror
Because complications or the potential for com- fusionis. Another relentless form of diplopia is
plications are an unavoidable part of strabismus sur- caused by bilateral cranial nerve palsies producing
gery, guidelines for treating and/or avoiding these secondary deviations in all fields of gaze and making
complications should be established. Each surgeon comfortable fusion impossible. Further active treat-
should acquire and maintain sufficient skills. This is a ment may only worsen that problem and the patient
must. The surgeon should have sufficient knowl- should be counseled appropriately. Patching one eye
edge of his patient, the reason for doing the surgery, or use of an opaque contact lens or, best of all, estab-
and should prepare both himself and the patient pre- lishing the patient's own suppression mechanism
operatively in light of the patient's specific needs. could be the only real remedy. Some of the most
The surgeon and his patient should then maintain unfortunate diplopia-plagued patients that I have
realistic expectations with regard to the outcome of encountered are those with acquired third cranial
the proposed strabismus surgery. nerve palsy, usually with aberrant regeneration, who
have their eyes fairly well straightened by surgery but
Informed consent who have constant and incapacitating double vision.
Preoperative informed consent obtained for stra- Unless these patients can develop suppression, which
bismus surgery should include these potential com- they often cannot, they may be better off unoperated
plications: loss of vision, diplopia, and need for reop- retaining a larger angle of strabismus or as an alterna-
eration. A long list of complications which could tive, with some form of occlusion.
include bleeding, infection, and anesthetic problems Some postoperative patients will literally look
need not be mentioned specifically. All are implied for diplopia and in the process become agitated.
by the three warnings given. These patients may complain of double vision when
When a complication is encountered, it is essen- reading while lying on their back in bed or while
tial to deal with the patient with candor and compas- assuming some other extreme position or when look-
sion, maintaining a healthy doctor-patient relation- ing in extremes of gaze. I tell these patients to
ship. Denial of a complication by the surgeon is guar- assume a more hygienic posture for reading and tel-
anteed to exacerbate the problem in the patient's evision viewing. It is useful to differentiate diplopia
mind. This is understandable. If the patient is having that must be looked for and found from diplopia
a problem and the surgeon does not recognize its exis- that looks for and finds the patient and in the
tence, the patient might magnify the problem until the process disrupts the normal flow of events. The for-
surgeon or some other physician (or attorney) does mer is an unavoidable part of much strabismus. It can
recognize a problem, either real or imagined. The be dealt with by the patient in most cases. The latter
best way to deal with a complication is to recognize may be dealt with by surgical or nonsurgical means
that its exists, convey this awareness to the patient, but in some cases could be intractable. For the most
and implement a plan to remedy the situation. part, patients seem satisfied by and benefit from this
explanation of double vision. Further, it is valuable to
Diplopia tell patients that anyone with two eyes, even those
with perfectly normal motility, can experience double
Bothersome diplopia after surgery is a problem vision in certain circumstances. Some patients with
that can be treated successfully temporarily and in longstanding horizontal strabismus and no preopera-
most cases, permanently. The immediate remedy for tive fusion potential experience diplopia after their
diplopia is obvious: patch one eye constantly or alter- eyes are aligned by surgery. When these patients
nate the patch between the two eyes. This type of complain about diplopia, you can offer to put the eyes
treatment is often appropriate for the patient who has back in the preoperative state at no charge if the
an early postoperative overcorrection such as diplopia is more of a detriment than the alignment is
esotropia after surgical treatment for an intermittent beneficial. This would be said with tongue in cheek
exotropia. Diplopia persisting more than a few days and with the assurance that in nearly every case the
may require treatment with prisms, either temporary diplopia goes away with onset of suppression provid-
Fresnel prisms or permanent prisms ground into the ed the patient will give the process sufficient time. I
spectacles. If time and these remedies fail, reopera- know of no patient to date who has exercised this
tion to relieve the diplopia may be necessary. option.
457
Chapter 17
458
Complications of strabismus surgery
Figure 1
A Acute allergic suture reaction occurred 12 days after
inferior rectus resection.
B After application of prednisolone 0.12% twice a day for 10
days, the reaction disappeared.
459
Chapter 17
Treatment
Treatment consists of topically applied steroids; I
prefer prednisolone 0.12% twice a day for 2 weeks. If
the mass persists it must be surgically excised.
Repeat strabismus surgery may be required at this
time.
Figure 3
Mild, acute suture reaction, presumed allergic, after muscle
reattachment with 6-0 Vicryl suture. This occurs rarely.
Subconjunctival cysts
A subconjunctival cyst may occur when small
B segments of the conjunctival epithelium are buried at
the time of the conjunctival wound closure. The cysts
are usually 2 to 3 mm in diameter and are filled with
a clear fluid. They are cosmetically objectionable but
do not ordinarily compromise the results of strabis-
mus surgery. Rarely the cysts will be very large and
extend back into the orbit for 10 mm or more, occa-
sionally affecting motility (Figure 4).
Figure 2 Prevention
A Chronic suture granuloma persisted 6 months after Careful closure of the conjunctiva at the time of
resection of the lateral rectus.
B The eye looks white and the lateral conjunctiva smooth surgery will prevent cysts.
after excision of the granuloma.
460
Complications of strabismus surgery
Treatment
Small anterior subconjunctival cysts may be made to excise the entire epithelial lining. Larger,
removed in the office under topical or subconjuncti- orbital cysts are removed in the operating room. In
val 1% lidocaine (Xylocaine) anesthesia. I prefer my experience, these cysts are always outside the
total excision of these cysts intact, if possible. If the muscle cone and therefore can be approached without
cyst ruptures during dissection, an attempt should be disrupting important orbital contents.
A B
C D
Figure 4
A A subconjunctival cyst after medial rectus recession. D CT scan demonstrating bilateral cysts over the medial
B Large subconjunctival-orbital cyst over the left medial recti extending deeply into the orbit.
rectus E The subconjunctival-orbital cyst over the left medial
C Close up view of cyst, left eye. rectus, seen at the time of surgery for its removal.
461
Chapter 17
Figure 5
A Prolapsed Tenon's capsule persisted 2 weeks B Tenon's capsule has retracted without treatment three
postoperatively. months after surgery.
462
Complications of strabismus surgery
Suture abscess
A suture abscess appears as a yellowish elevation delle different from an ulcer or corneal melt. The
over the suture placement site. It usually occurs with- localized drying leading to delle formation is usually
in the first week postoperatively. The eye is deeply caused by elevation of the conjunctiva at the limbus.
injected and a purulent drainage may be present. An Delle were found in 8% of 100 consecutive patients
abscess occurs more often when a nonabsorbable who had extraocular muscle surgery with the limbal
suture such as Merseline has been used. This com- approach. Most were subtle actually subclinical seen
plication is rare. only with the slit lamp for this study. Delle are usu-
ally benign complications that do not affect the out-
Prevention come of strabismus surgery. Clinically significant
Aseptic technique at the time of surgery and rou- delle are rare.
tine use of antibiotics postoperatively will prevent
suture abscess. Prevention
Smooth closure of the conjunctiva, especially
Treatment adjacent to the limbus, will prevent delle formation.
Treatment includes drainage of the abscess under If decreased tear formation is found preoperatively,
topical, local, or general anesthesia, removal of the the operated eye should be patched and/or artificial
suture nidus if present, and appropriate topical antibi- tears used after surgery.
otic treatment after a culture has been obtained.
Delle Treatment
A delle is a small area of corneal stromal thin- Occlusion of the eye for 1 or 2 days will result in
ning caused by localized drying of the cornea. This rehydration of the cornea and disappearance of the
corneal thinning does not represent melting away of delle. Frequent instillation of lubricating drops may
tissue, but rather shrinkage of tissue as a result of be used in lieu of a patch. If the conjunctival eleva-
local dehydration. The corneal epithelium is intact tion causing the dellen persists, the conjunctiva is
and does not stain, but fluorescein will pool in the smoothed surgically or the offending conjunctiva can
area giving the appearance of staining. The intact be excised with conjunctival recession and bare scle-
epithelium along with stromal thinning makes the ra closure.
A B
Figure 6
A Rather large delle with associated stromal clouding. This C The cause of a delle is localized corneal stromal
clouding is reversible. dehydration caused by a mound of conjunctiva at the
B A less obvious delle adjacent to an area of elevated limbus, disrupting lid-cornea apposition.
conjunctiva.
463
Chapter 17
Figure 7
A Ptosis of the left upper lid occurred after excessive B Ptosis of the right lower lid occurred after a 5 mm
resection of the left superior rectus. The surgeon recession of the right inferior rectus without sufficient
actually intended to resect the left lateral rectus. freeing of the inferior rectus from its surrounding
structures.
continued.
464
Complications of strabismus surgery
Prevention
Expose and hook the superior oblique tendon
under direct vision.
Treatment
Repair of the medial horn of the levator aponeu-
rosis is made after a skin incision.
Scleral perforation
The true incidence of inadvertent scleral perfora-
tion occurring during extraocular muscle surgery is B
unknown. However, it has been estimated to occur in
from 8% to 12% of patients in series reported before
the advent of smaller caliber needles. In a recent
prospective series where the retina at the site of mus-
cle reattachment was examined before and after mus-
cle surgery, one scleral perforation was identified in
194 procedures done in 144 eyes. This was noted at
the time of surgery as a small retinal hemorrhage
observed with the indirect ophthalmoscope in the reti-
nal periphery at a point corresponding to the muscle
reattachment. No treatment was given. Six months
Figure 8
later a small chorioretinal scar was noted. I have con- A The peripheral retina of the right and left eyes of a 35-
firmed a retinal perforation at the time of surgery six year-old patient who had bilateral lateral rectus
times. Twice I observed a small bead of vitreous on recession 25 years before.
the sclera at the site of needle placement in the scle- B These retinal scars are adjacent to the presumed site of
muscle reattachment and are thought to be caused by
ra. Observation of the retina in the operating room scleral and retinal perforation at the time of surgery. No
revealed a dot hemorrhage. No treatment was given treatment for this was given at the time. Visual acuity is
for these cases or the other four. A small chorioreti- 20/20 in each eye. The patient was not aware of any
problem with her eyes.
465
Chapter 17
Prevention
Needles should be placed in sclera with a short the postoperative period. The slipped muscle remains
shallow track with the widest dimension of the needle attached to sclera but it slips back, usually in its cap-
parallel to sclera. If a needle is put in sclera at an sule. This phenomenon has been called stretched
angle or on edge, scleral perforation could occur. In scar by Ludwig.
cases of reoperations with adhesions and the case of A lost muscle is most likely to occur with the
thin sclera, careful sharp dissection should be used. medial rectus muscle because this muscle is not asso-
In cases where thin sclera is suspected because of sys- ciated with other muscles or orbital structures.
temic connective tissue disease or high myopia, mar- Loss of the medial rectus muscle can occur when
ginal myotomy may be performed as a primary weak- extensive dissection of the intermuscular membrane
ening procedure to avoid the risk of scleral perfora- has been done and the surgeon simply loses hold of
tion associated with needle placement for recession. the muscle and it retracts behind Tenon's into the fat
A resection may be carried out safely in such cases by compartment. This could also happen if the sutures
leaving a slightly longer stump at the insertion and attaching the muscle to sclera fail immediately after
using it for muscle reattachment. The muscle stump surgery and before tissue union takes place. I have
may also be used as a safe anchor for a hang-back not experienced this so can only guess what takes
recession. place intra-operatively. Given the relationship of the
lateral rectus to the inferior oblique, the inferior rec-
Treatment tus to Lockwood's, and the superior rectus to the
Any time scleral perforation is suspected at the superior oblique tendon, it is unlikely that these mus-
time of surgery, the patient's pupil should be dilated cles would be lost from view at the time of surgery.
and the retina over the site of suspected perforation In the event a lost muscle is not detected at the time
should be examined using an indirect ophthalmo- of surgery, it will become obvious in the immediate
scope. Some surgeons prefer to treat inadvertent scle- post operative period after the suture breaks, unties,
ral perforations with prophylactic application of or the attachment to the muscle or sclera fails. The
cryotherapy, diathermy, or even a scleral buckle for eye in this case will not move in the field of the lost
support. I strongly disagree with this approach. muscle.
In my opinion, simple perforation without pro- Slipped muscles are not at all uncommon. They
lapse of vitreous or uvea should be left untreated. If tend to occur in the weeks, months, or years after sur-
uvea or vitreous prolapses or if the defect is large, it gery. This is accompanied by a gradual over-correc-
should be closed with sutures, with or without a scle- tion in the case of a slipped recessed muscle or a grad-
ral graft, and further prophylactic treatment to the ual under correction in the case of a slipped resected
retina should be considered and performed by a reti- muscle. There will also be diminished ductions in the
na specialist. If further manipulation of the eye mus- case of a slipped muscle.
cle would create a hazardous situation for the eye, the
extraocular muscle procedure should be suspended at
Prevention
that time. I suspect that in cases of retinal perforation The preventing of slipped or lost muscles
more harm has resulted from over-treatment than demands proper technique, requiring the surgeon to
from under-treatment. Sprunger treated scleral- do the following:
retinal perforation created in a rabbit with cryo and 1. Place sutures securely into muscle or tendon
laser treatment. The amount of reaction with cryo tissue. This is best accomplished by placing
was significantly more than that created by a ring of the suture 0.5 mm to 1.0 mm behind the
diode laser. However, experience tells us that no insertion of the muscle during recession and a
treatment is the safest and most effective. like amount behind the muscle clamp or
crimped line during resection. This resec-
Slipped or lost muscle tion effect is inconsequential in my experi-
There is a significant difference between a ence because the surgeon who pays careful
slipped muscle and a so-called lost muscle. A lost attention to the results of his/her surgery will
muscle is not really lost. It simply is no longer con- adjust surgical numbers to the technique
nected to sclera and has disappeared from view. The employed.
surgeon knows where the muscle is. It is in the orbit, 2. Place the needle into sclera producing a track
but it cannot be seen! The events surrounding a lost that is at least 1.5 mm long, including super-
muscle usually occur at the time of surgery and rep- ficial scleral fibers and at least 0.2 mm deep.
resent an intraoperative complication. In contrast, a 3. Use at least 6-0 synthetic absorbable suture
slipped muscle tends to occur gradually over time in tied with a double overhand knot with a
square knot on top (surgeon's knot).
466
Complications of strabismus surgery
4. Before securing the muscle to sclera after When a lost muscle is later suspected, a useful
recession or resection, limit the dissection of diagnostic technique is computerized tomography or
intermuscular membrane to a point anterior to MRI. If the muscle is seen behind posterior Tenon's
the emergence of the muscle through posteri- capsule, careful dissection can be carried out to iden-
or Tenon's capsule, thereby limiting the tify the muscle that can then be reattached to the scle-
extent of potential posterior slippage of the ra. I have retrieved a medial rectus muscle that had
muscle and ensuring that the muscles cut end been lost many years before by asking the patient
will stay visible even if attachment to the during a procedure done with local anesthesia to
globe by suture is lost. adduct the eye while I explored the medial sub-
Tenon's space. A dimple in Tenon's that appeared
Treatment during attempted adduction led to the muscle which
Treatment of a lost muscle in the operating was identified, dissected free, and reattached to the
room has been discussed above. Apparent detach- globe successfully. However, the surgeon should be
ment of a muscle occurring immediately after the cautioned against carrying out extensive blind
patient has left the operating room is an indication for exploration and grasping in search of a lost muscle,
immediate return to the operating room. At this time, if this dissection produces fat herniation and exces-
careful search for the muscle should be carried out. sive bleeding. This can cause irreparable damage,
This can be aided by finding the disrupted suture. If affecting alignment and conjunctival appearance. If
the muscle is found, it is re-sutured at the intended considerable difficulty is anticipated in finding a
point. Several drops of Neo-Synepherine 2.5% lost muscle, it may be better to carry out a suitable
placed on the operative site will blanch Tenon's and extraocular muscle transfer, or stop and seek immedi-
episclera and will make the red muscle tissue more ate help or refer the patient.
evident.
Figure 9
A A 6-year-old boy after having undergone a recession of B The left eye fails to reach the midline in levoversion.
the left medial rectus muscle and a resection of the left C The same patient after reattachment of the left lateral
lateral rectus muscle. A diagnosis of a lost left lateral rectus.
rectus muscle was made.
467
Chapter 17
Figure 10
The CT scan of the right medial rectus muscle that has slipped in its capsule.
Note by the position of the lens that the right eye is exotropic.
Figure 11
A This patient has a slipped left medial rectus muscle after bimedial rectus recession.
Note the widened palpebral fissure on the left side during attempted adduction.
B The right medial rectus was lost when it slipped off a resection clamp. Prolonged blind
search for the muscle was unsuccessful and produced a large prolapse of orbital fat.
468
Complications of strabismus surgery
469
Chapter 17
Treatment
The inferior oblique muscle must be explored
and re-weakened using the surgeon's preferred tech-
nique. The inferior oblique traction test is a useful
means for confirming persistent inferior oblique con-
nections (see chapter 4).
Prevention
Persistent overaction of the Inferior oblique adherence syndrome can be
inferior oblique muscle avoided if care is taken at the time of surgery. The
Persistent overaction of the inferior oblique mus- inferior oblique muscle should be engaged under
cle may occur if some of the inferior oblique fibers direct visualization. A small muscle hook should be
have been left intact. It may also occur if the severed placed carefully behind the inferior oblique muscle
ends of a myectomized muscle rejoin by muscle or and not simply thrust deeply into the orbit. The inter-
fibrous tissue. In other cases, the proximal end of the muscular membrane (posterior Tenon's capsule)
inferior oblique can attach to sclera, so as to allow should be left intact and any bleeding should be con-
considerable inferior oblique function. trolled with carefully applied cautery. If any orbital
fat is encountered, it should be reposited behind the
Prevention intermuscular membrane (posterior Tenon's capsule)
Careful exposure of the posterior aspect of the and the defect closed with several 8-0 Vicryl sutures.
inferior oblique muscle should be carried out routine-
ly. Any remaining fibers should then be transected.
Treatment
This technique must be applied for both myectomy The treatment of inferior oblique adherence syn-
and recession. It has been said that the inferior drome presents a challenge. The surgical area should
oblique muscle can have two or three heads. be dissected carefully and adhesions lysed until pas-
However, this possible anatomic variation is of no sive ductions are free. Appropriate yoke muscle sur-
significance in the case of myectomy performed in gery may be performed but persistence of some
the inferior temporal quadrant. When a myectomy is restriction is the rule, in spite of treatment.
470
Complications of strabismus surgery
Figure 13
Inferior oblique adherence, left eye. The left eye has limited
elevation, depression, and adduction. The latter leads to the
increased abduction in elevation creating a V pattern.
A B
Figure 14
A J anomaly with the inferior oblique attached to the lateral B The inferior oblique is inserted at the inferior aspect of
rectus at the insertion of the lateral rectus. This the insertion of the lateral rectus that had been resected
complication produces various expressions of an at the previous surgery.
acquired vertical strabismus after surgery on the lateral
rectus muscle. continued.
471
Chapter 17
C D
Muscle-tendon rupture
During the course of extraocular muscle surgery, the muscle lost and carrying out a muscle transfer
a muscle or tendon can rupture. This may be caused could be the best course in case of such a lost hori-
by excessive force applied to the normal muscle zontal rectus muscle, especially the medial.
while handling it on a muscle hook or because the
muscle or tendon is abnormally thin or atrophic.
Prevention
Greenwald has reported rupture in several patients. I Rupture of an extraocular muscle or tendon can
observed rupture of the inferior oblique while an be avoided by limiting the force applied to an
assistant was holding the muscle with two muscle extraocular muscle or tendon during manipulation in
hooks before I was to have placed hemostats before the course of strabismus surgery. In case an abnor-
cutting out a 5 mm segment of muscle. The assistant mally thin or atrophic muscle is suspected or
could not explain why this happened to the healthy observed, extra caution must be exercised.
muscle. We believed that the inexperienced assistant
simply pulled too hard. A minor amount of bleeding Hyphema
was controlled and the distal end of the muscle was I encountered hyphema one time during strabis-
trimmed. The proximal muscle disappeared behind mus surgery. This complication occurred after tuck-
Tenons. Unfortunately, this patient had limited ele- ing a superior oblique tendon. The patient had under-
vation in adduction on a mechanical basis which was gone cataract surgery one year earlier. It was thought
refractory to treatment. Bleeding with tissue damage that an abnormal iris vessel in the superior anterior
in the area of Lockwood's ligament was the suspected chamber angle associated with the cataract incision
cause. In another case, the superior oblique tendon had been ruptured during manipulation of the globe.
was pulled from the globe while the tendon was being The hyphema cleared in 24 hours without complica-
tucked. The tuck was then converted to a resection tion.
without complication. In cases where excessive force
is the reason for rupture, as occurred with the inferior Prevention
oblique described earlier, trauma to associated struc-
There is probably no sure way to prevent the
tures may lead to an adherence syndrome. If the hor-
development of hyphema. However, when perform-
izontal recti are ruptured resulting in retraction of the
ing eye muscle surgery on a patient who has had prior
proximal portion into the posterior fat compartment,
cataract surgery, I exercise great care while manipu-
excessive search should be avoided to prevent further
lating the muscles and globe.
trauma leading to restrictive strabismus. Declaring
472
Complications of strabismus surgery
473
Chapter 17
Figure 15
Brown syndrome after superior oblique tuck before and after take down.
Prevention
The surest way to prevent Brown syndrome post- of my attempt to classify superior oblique palsy into
operatively is to avoid surgery to shorten the superior congenital palsy with an abnormal tendon and
oblique tendon. The next best way is to evaluate the acquired with a normal tendon.
superior oblique tendon carefully before shortening
it. This is done by means of forced ductions compar- Treatment
ing the two sides and by inspection of the tendon for When Brown syndrome is encountered postoper-
location of the insertion and for redundancy of the atively, time is the first consideration. The patient is
tendon. It is a good idea to observe the normal supe- advised to look up in adduction with the involved eye.
rior oblique tendon when enucleating an eye. If after several weeks to months the restriction per-
Knowledge of what the normal superior oblique ten- sists and annoys the patient, the tuck can be taken
don looks and feels like provides a useful background down or the resected tendon can be disinserted or
for evaluating and grading the abnormal tendon. Of recessed. In most cases, this second procedure will
course, excess manipulation should be avoided when correct the problem without seriously compromising
enucleating an eye with malignancy. If a superior the results of the original surgery.
oblique tendon shortening procedure is performed,
repeat intraoperative forced ductions should also be Symblepharon
performed. At the conclusion of the procedure a suc-
cessfully tucked tendon will allow full or nearly full Symblepharon may occur with improperly
elevation in adduction with very little increase in placed conjunctival incisions (Figure 16).
resistance. Prevention
Early in my career, I tucked a superior oblique
tendon 22 mm. This produced a perfect result. Careful conjunctival incision and closure will
Subsequently, I produced a severe Brown syndrome prevent symblepharon.
in 17 of 59 patients undergoing superior oblique
shortening. Nine of these patients required surgical Treatment
take down of the tuck. As a result of this experi- Conjunctival recession with bare sclera closure
ence, I began to look more critically at the differences should be carried out if ocular motility is restricted or
in the superior oblique tendon. This was the genesis if the conjunctiva is reddened and unsightly.
474
Complications of strabismus surgery
Treatment
A severed vortex vein should be controlled with
local pressure over the bleeding site. Cautery may be
used. The treatment of diffuse orbital hemorrhage
from blood dyscrasia depends on the surgeon's suc-
cess at maintaining a reasonable intraocular pressure
during the acute period. Assistance from a hematolo-
gist is useful and should be sought. Fortunately, chil-
dren and young adults can withstand brief periods (up
to several hours) of very high intraocular pressure
without sustaining damage. If such a hemorrhage
occurs, osmotic agents and digital massage along
with careful monitoring of the intraocular pressure
are indicated. Such hemorrhage occurring after
extraocular muscle surgery should not be treated with
Figure 16 Symblepharon paracentesis.
Orbital hemorrhage
Orbital hemorrhage may occur after a vortex
vein is cut or when a patient has an unrecognized
blood dyscrasia. Cutting a vortex vein causes a large,
usually anterior, hematoma with dark blood that
results in unsightly lid swelling and discoloration.
Blood dyscrasias cause a far more serious generalized
oozing into all orbital tissue. In one operation I did A
which resulted in generalized orbital hemorrhage, 8
mm of proptosis occurred in both eyes along with
intraocular pressure elevation to 50 mm Hg, corneal
edema, and easily induced retinal artery pulsations
(Figure 17).
Prevention
Careful dissection coupled with awareness of the
location of the vortex veins can reduce, if not elimi-
nate, hemorrhage. Blood dyscrasias should be uncov-
ered preoperatively in the course of securing an ade-
quate history. In any case where a bleeding tendency
is suspected, hematologic evaluation should be
obtained. Preoperative use of aspirin can cause a
decrease in platelets which in turn will promote
bleeding during and after surgery. Patients should
stop taking aspirin for 1 or 2 weeks before surgery. If
a patient is on anticoagulant medication, stopping or
reducing this medication should be discussed with the Figure 17
primary care physician. I have operated safely on A A 28-year-old man with 70+ prism diopters of exotropia
many patients using anticoagulants without complica- underwent a 7-mm recession of both lateral rectus
muscles and an 8-mm resection of both medial rectus
tion. In these cases special attention was given to muscles.
hemostasis with meticulous application of wet field continued.
cautery.
I have also operated successfully on an adult
patient with hemophilia after he was prepared with
preserved globulin by his hematologist.
475
Chapter 17
476
Complications of strabismus surgery
477
Chapter 17
Unacceptable overcorrections
and undercorrections
Undesirable overcorrections and undercorrec- correction or undercorrection I mean more or less
tions are an inevitable accompaniment of strabismus correction than the surgeon intended. This is signifi-
surgery. cant because some categories of patients should be
undercorrected relative to ortho position and others
Prevention should be overcorrected.
A careful, accurate workup, correct choice of
surgery, and proper execution of surgery will reduce a
Treatment
surgeon's unacceptable overcorrections and undercor- Overcorrections or undercorrections should be
rections. The percentage of cases corrected to within treated according to Cooper's dictum; that is, as
10 prism diopters of the intended postoperative though they were new cases with appropriate med-
angle will depend on multiple factors covered in this ical, optical, orthoptic, or surgical remedies instituted.
book and also on the surgeon's ability to learn from In addition, the surgeon should rely on careful meas-
experience. Because similar types of patients react in urements, force and velocity studies, and on findings
similar ways, the surgeon should not make the same at surgery when doing secondary surgery.
mistake repeatedly, but instead learn from his
patients. It should also be emphasized that by over-
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